MyEClinic
MyEClinic
Health Dictionary Find a Doctor

Eosinophilic granulomatosis with polyangiitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]

Synonyms and keywords: Allergic granulomatosis; eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

Eosinophilic granulomatosis with polyangiitis previously called Churg-Strauss syndrome is a small and medium-sized necrotizing vasculitis, with extravascular granuloma formation. The disease was first described by Churg and Strauss in 1951. The etiology is not known. However, various environmental factors, allergens, genetics, and drugs may play a role in triggering disease process by activating eosinophils, B and T lymphocytes and macrophages. The disease is characterized by the presence of asthma, peripheral eosinophilia, rhinosinusitis, peripheral neuropathy and multiple organ involvements including skin, GI tract, and kidney.

Historical Perspective

Two pathologists Churg and Strauss first described the disease in 1951. They named it as allergic granulomatosis with angiitis. Over the years, they called the disease by the name of Churg-Strauss syndrome. In 2010, American College of Rheumatology changed this name to eosinophilic granulomatosis with polyangiitis based on histopathology.

Classification

Revised International Chapel Hill Consensus Conference 2012 on nomenclature of vasculitides, defines eosinophilic granulomatosis with polyangiitis (formerly known as Churg – Strauss syndrome) as an eosinophilic, granulomatous inflammatory disease affecting most commonly the conducting pulmonary airways, and leading to a necrosis of the small and/or medium-sized vessels. Eosinophilic granulomatosis with polyangiitis is often synonymous with adult-onset asthma. According to revised CHCC 2012, eosinophilic granulomatosis with polyangiitis is considered as a variant of the ANCA – associated vasculitis.

Pathophysiology

Eosinophilic granulomatosis with polyangiitis is a medium and small vessel vasculitis, leading to necrosis. The pathogenesis of eosinophilic granulomatosis with polyangiitis is not fully understood. Eosinophilic granulomatosis with polyangiitis occurs as a result of a complex interaction involving genetic and environmental factors that lead to an inflammatory response involving eosinophils, lymphocytes. Autoimmunity has an evident role in the presence of ANCA, hypergammaglobulinemia, elevated levels of immunoglobulin E, and rheumatic factor in the pathogenesis. HLA-DRB4 is correlated with increased risk of development of vascular manifestations of the Churg-Strauss syndrome. On microscopic pathology, eosinophilic infiltration, necrotizing granulomatous vasculitis and necrosis of small and medium-sized arteries can be seen.

Causes

The etiology of eosinophilic granulomatosis with polyangiitis is not known. Various allergens, infections, vaccinations and drugs may be responsible for developing disease through an allergic or autoimmune response. Genetics may play a role includes, HLA -DRB4 and IL-10 gene polymorphisms are associated with the development of eosinophilic granulomatosis with polyangiitis.

Differentiating Churg-Strauss syndrome from Other Diseases

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that can cause eosinophilia, purpura, alveolar hemorrhage, necrotizing extra-capillary glomerulonephritis, such as granulomatosis with polyangiitis and microscopic polyangiitis.

Epidemiology and Demographics

The incidence of Eosinophilic granulomatosis with polyangiitis range from 2.5 to 16.6 per 100,000 individuals. Prevalence ranges from 2 to 16 per 100,000 individuals. Mean age at diagnosis of eosinophilic granulomatosis with polyangiitis around 45-50 years. In general, eosinophilic granulomatosis with polyangiitis affects men and women equally.

Risk Factors

There are no established risk factors for eosinophilic granulomatosis with polyangiitis. However, certain environmental agents including various allergens, infections, desensitization, vaccination and genetics may act as triggering agents. Those triggers may be responsible for the inflammatory response with eosinophils and lymphocytes.

Natural History, Complications and Prognosis

Eosinophilic granulomatosis with polyangiitis develops through three phases, include prodromal phase, eosinophilic phase, vasculitic phase. Most complications result from the vasculitic phase. Most common complications include cardiomyopathy, myocardial infarction, perimyocarditis, rapidly progressive renal failure, GI bleeding, neuropathy and status asthmaticus. Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated. Prognosis is most likely dependent on stage at which the disease was diagnosed and organ involvement. The five-factor score assessment (FFS) is a good predictor of survival rate. It can be used to choose the appropriate treatment.

Diagnosis

History and Symptoms

Obtaining a complete history is an important aspect in making a diagnosis of eosinophilic granulomatosis with polyangiitis. As it can help differentiate between the ANCA associated vasculitis and other possible causes that may mimic the disease. Symptoms of eosinophilic granulomatosis with polyangiitis typically develops through three phases, include prodromal phase, eosinophilic phase, and vasculitis phase. Clinical presentation depends on organ system involvement. Most common symptoms include asthma, sinusitis, weakness, arthralgia, purpura, arrythmias, hematuria and peripheral neuropathy.

Physical Examination

A comprehensive physical examination including pulmonary, ENT, neurologic, skin, abdominal and renal systems must be performed to help identify and properly diagnose eosinophilic granulomatosis with polyangiitis form other diseases. On examination, patients may show clinical manifestations of asthma (dyspnea, tachypnea), petechiae, palpable purpura, skin nodules, rhinitis, nasal polyposis, chest pain, abdominal pain, and neurologic manifestations. On auscultation, wheezing, rhonchi, friction rub, abnormal heart sounds may be found.

Laboratory Findings

The laboratory findings in eosinophilic granulomatosis with polyangiitis  include complete blood count with differntial to evaluate abnormal eosinophilia, serologic and immunologic tests to identify antineutrophil cytoplasmic antibodies, acute phase reactants, and urinalysis to evaluate proteinuria and microscopic hematuria. Gold standard for diagnosis is biopsy of lung.

Chest X Ray

On chest x-ray, eosinophilic granulomatosis with polyangiitis is characterized by bilateral multifocal nonsegmental consolidation, bronchial wall thickening, reticulonodular opacities, bilateral hilar adenopathy, and pleural effusion.

CT Scan

On high-resolution computerized tomography (HRCT) scan, eosinophilic granulomatosis with polyangiitis will show airspace consolidation, ground-glass opacities, centrilobular nodules, bronchial wall thickening and/or dilatation, pleural effusions, and hilar or mediastinal lymph node enlargement.

Ultrasound

There are no significant ultrasound findings associated with eosinophilic granulomatosis with polyangiitis.

Other Imaging Findings

Electrocardiogram and echocardiogram, GI endoscopy, electromyelography can be used to diagnose eosinophilic granulomatosis with polyangiitis.

Treatment

Medical Therapy

The mainstay of management for eosinophilic granulomatosis with polyangiitis is glucocorticoids and cyclophosphamide. Immunosuppressive agents (eg, azathioprine and methotrexate) can be used for maintenance therapy. Rituximab, interferon-alpha, anti IgE antibodies and plasma exchange can be used as second-line therapy in the management of eosinophilic granulomatosis with polyangiitis.

Surgery

Surgical intervention is not usually recommended for the management of eosinophilic granulomatosis with polyangiitis. However, myringotomy, polypectomy, and endoscopic sinus surgery may be performed in some patients to treat upper airway disease.

Prevention

There are no established measures for the primary and secondary prevention of eosinophilic granulomatosis with polyangiitis.

References

Template:WH Template:WS

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Chandrakala Yannam, MD [4]

Overview

Two pathologists Churg and Strauss first described disease in 1951. They named it as allergic granulomatosis with angiitis. Over the years, they called the disease by the name of Churg-Strauss syndome. In 2010, American college of rheumatology changed this name to eosinophilic granulomatosis with polyangiitis based on histopathology.

Historical Perspective

  • In 1951, Russian born American pathologist Jacob Churg along with German born, American pathologist Lotte Strauss first described the disease as allergic granulomatosis, allergic angiitis, and periarteritis nodosa.[1][2][3]
  • The name ‘Churg-Strauss syndrome has’ been changed to ‘Eosinophilic granulomatosis with polyangiitis’ in 2010 by the American College of Rheumatology, and the European League Against Rheumatism. The name has been changed to characterize the pathology of the disease rather than having it remain as a historical reference. [4]

References

  1. ↑ CHURG J, STRAUSS L (1951). “Allergic granulomatosis, allergic angiitis, and periarteritis nodosa”. Am J Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.
  2. ↑ Template:WhoNamedIt
  3. ↑ Churg J, Strauss L (1951). “Allergic granulomatosis, allergic angiitis, and periarteritis nodosa”. Am. J. Pathol. 27 (2): 277–301. PMID 14819261.
  4. ↑ Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC; et al. (2011). “Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis”. Arthritis Rheum. 63 (4): 863–4. doi:10.1002/art.30286. PMID 21374588.

Template:WH Template:WS

Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]

Overview

Revised international chapel hill consensus conference 2012 on nomenclature of vasculitides, defines eosinophilic granulomatosis with polyangiitis (formerly known as Churg – Strauss syndrome) as an eosinophilic, granulomatous inflammatory disease affecting most commonly the conducting pulmonary airways, and leading to a necrosis of the small and/or medium sized vessels. Eosinophilic granulomatosis with polyangiitis is often synonymous with adult-onset asthma. According to revised CHCC 2012, eosinophilic granulomatosis with polyangiitis is considered as a variant of the ANCA – associated vasculitides.

Classification

American College of Rheumatology (ACR) Classification of Eosinophilic granulomatosis with polyangiitis
Asthma
Eosinophilia (>10% of total WBC)
Neuropathy (mononeuropathy or polyneuropathy)
Pulmonary infiltrate
Paranasal sinus abnormality
Biopsy that shows extravascular eosinophil infiltration

References

  1. ↑ Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (January 2013). “2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides”. Arthritis Rheum. 65 (1): 1–11. doi:10.1002/art.37715. PMID 23045170.
  2. ↑ Mouthon L, Dunogue B, Guillevin L (2014). “Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome)”. J. Autoimmun. 48-49: 99–103. doi:10.1016/j.jaut.2014.01.018. PMID 24530234.
  3. ↑ Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP; et al. (1990). “The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)”. Arthritis Rheum. 33 (8): 1094–100. PMID 2202307.
  4. ↑ Gioffredi A, Maritati F, Oliva E, Buzio C (2014). “Eosinophilic granulomatosis with polyangiitis: an overview”. Front Immunol. 5: 549. doi:10.3389/fimmu.2014.00549. PMC 4217511. PMID 25404930.

Template:WH Template:WS

Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]

Overview

Eosinophilic granulomatosis with polyangiitis is a medium and small vessel vasculitis, leading to necrosis and vasculitis. The pathogenesis of eosinophilic granulomatosis with polyangiitis is not fully understood. It may occur as a  result of a complex interaction involving genetic and environmental factors that lead to an inflammatory response involving eosinophils, lymphocytes and giant cells. Autoimmunity has an evident role in the presence of ANCA, hypergammaglobulinemia, elevated levels of immunoglobulin E, and rheumatic factor in the pathogenesis. HLA-DRB4 is correlated with increased risk of development of vascular manifestations of the churg-strauss syndrome. On microscopic pathology, eosinophlic infiltration, necrotizing granulomas vasculitis and necrosis of small and medium-sized arteries can be seen.

Pathophysiology

Pathogenesis

Genetics

Associated Conditions

The following conditions are associated with Eosinophilic granulomatosis with polyangiitis:[8][9][10][11]

Gross Pathology

On gross pathology, the following changes are seen in patients with eosinophilic granulomatosis with polyangiitis include:[12]

Microscopic Pathology

On microscopic examination, following findings can be seen in eosinophilic granulomatosis with polyangiitis, include:[13][14]

Lung biopsy showing eosinophilic vasculitis consistent with Churg-Strauss syndrome – By By Nephron – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=15244866


References

  1. ↑ Hellmich B, Ehlers S, Csernok E, Gross WL (2003). “Update on the pathogenesis of Churg-Strauss syndrome”. Clin. Exp. Rheumatol. 21 (6 Suppl 32): S69–77. PMID 14740430.
  2. ↑ Tsurikisawa N, Saito H, Tsuburai T, Oshikata C, Ono E, Mitomi H, Akiyama K (September 2008). “Differences in regulatory T cells between Churg-Strauss syndrome and chronic eosinophilic pneumonia with asthma”. J. Allergy Clin. Immunol. 122 (3): 610–6. doi:10.1016/j.jaci.2008.05.040. PMID 18586318.
  3. ↑ Wechsler ME, Garpestad E, Flier SR, Kocher O, Weiland DA, Polito AJ, Klinek MM, Bigby TD, Wong GA, Helmers RA, Drazen JM (February 1998). “Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast”. JAMA. 279 (6): 455–7. PMID 9466639.
  4. ↑ Tuggey JM, Hosker HS (September 2000). “Churg-Strauss syndrome associated with montelukast therapy”. Thorax. 55 (9): 805–6. PMC 1745850. PMID 10950903.
  5. ↑ Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM (September 2007). “HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome”. Arthritis Rheum. 56 (9): 3159–66. doi:10.1002/art.22834. PMID 17763415.
  6. ↑ Bottero P, Motta F, Bonini M, Vecchio F, Ierna F, Cuppari I, Sinico RA (2014). “Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome?”. ISRN Rheumatol. 2014: 843804. doi:10.1155/2014/843804. PMC 3963189. PMID 24734195.
  7. ↑ Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT (June 2008). “Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener’s granulomatosis”. Arthritis Rheum. 58 (6): 1839–48. doi:10.1002/art.23496. PMID 18512809.
  8. ↑ Lee SH, Roh MR, Jee H, Chung KY, Jung JY (2011). “Wells’ syndrome associated with churg-strauss syndrome”. Ann Dermatol. 23 (4): 497–500. doi:10.5021/ad.2011.23.4.497. PMC 3229945. PMID 22148019.
  9. ↑ Maamar M, Tazi-Mezalek Z, Harmouche H, El Hamany Z, Adnaoui M, Aouni M (2012). “Churg-Strauss syndrome associated with AA amyloidosis: a case report”. Pan Afr Med J. 12: 30. PMC 3415051. PMID 22891088.
  10. ↑ Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, Reinhardt D, Wolf G, Hein G, Mall G, Schett G, Zwerina J (July 2009). “Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis”. Medicine (Baltimore). 88 (4): 236–43. doi:10.1097/MD.0b013e3181af35a5. PMID 19593229.
  11. ↑ Chumbley LC, Harrison EG, DeRemee RA (August 1977). “Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases”. Mayo Clin. Proc. 52 (8): 477–84. PMID 18640.
  12. ↑ CHURG J, STRAUSS L (1951). “Allergic granulomatosis, allergic angiitis, and periarteritis nodosa”. Am J Pathol. 27 (2): 277–301. PMC 1937314. PMID 14819261.
  13. ↑ Churg A (December 2001). “Recent advances in the diagnosis of Churg-Strauss syndrome”. Mod. Pathol. 14 (12): 1284–93. doi:10.1038/modpathol.3880475. PMID 11743052.
  14. ↑ Katzenstein AL (November 2000). “Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review”. Am. J. Clin. Pathol. 114 (5): 767–72. doi:10.1309/F3FW-J8EB-X913-G1RJ. PMID 11068552.

Template:WH Template:WS

Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

The etiology of eosinophilic granulomatosis with polyangiitis is not known. Various allergens, infections, vaccinations and drugs may act as a triggering agents, and are responsible for developing disease through an allergic or autoimmune response. Genetics may also play a role and HLA -DRB4 and IL-10 gene polymorphisms are associated with the development of eosinophilic granulomatosis with polyangiitis.

Causes

Life-threatening Causes

Causes

Genetic Causes

Causes by Organ System

Cardiovascular No underlying causes
Chemical/Poisoning No underlying causes
Dental No underlying causes
Dermatologic No underlying causes
Drugs Leukotriene receptor antagonists (eg, montelukast, zafirlukast), Anti IgE antibodies (eg, omalizumab), Mesalazine, PropylthiouracilMethimazole, silica, Cocaine
Ear Nose Throat No underlying causes
Endocrine No underlying causes
Environmental Allergens, vaccinations
Gastroenterologic No underlying causes
Genetic HLA – DRB4, HLA – DRB1*04 and *07, Interleukin -10 gene single nucleotide polymorphisms
Hematologic No underlying causes
Iatrogenic No underlying causes
Infectious Disease No underlying causes
Musculoskeletal/Orthopedic No underlying causes
Neurologic No underlying causes
Nutritional/Metabolic No underlying causes
Obstetric/Gynecologic No underlying causes
Oncologic No underlying causes
Ophthalmologic No underlying causes
Overdose/Toxicity No underlying causes
Psychiatric No underlying causes
Pulmonary No underlying causes
Renal/Electrolyte No underlying causes
Rheumatology/Immunology/Allergy No underlying causes
Sexual No underlying causes
Trauma No underlying causes
Urologic No underlying causes
Miscellaneous No underlying causes

Causes in Alphabetical Order

List the causes of the disease in alphabetical order.

References

  1. ↑ Hellmich B, Ehlers S, Csernok E, Gross WL (2003). “Update on the pathogenesis of Churg-Strauss syndrome”. Clin. Exp. Rheumatol. 21 (6 Suppl 32): S69–77. PMID 14740430.
  2. ↑ Safran T, Masckauchan M, Maj J, Green L (December 2017). “Wells syndrome secondary to influenza vaccination: A case report and review of the literature”. Hum Vaccin Immunother: 1–3. doi:10.1080/21645515.2017.1417714. PMID 29240526.
  3. ↑ Puéchal X, Rivereau P, Vinchon F (July 2008). “Churg-Strauss syndrome associated with omalizumab”. Eur. J. Intern. Med. 19 (5): 364–6. doi:10.1016/j.ejim.2007.09.001. PMID 18549941.
  4. ↑ Gómez-Puerta JA, Gedmintas L, Costenbader KH (October 2013). “The association between silica exposure and development of ANCA-associated vasculitis: systematic review and meta-analysis”. Autoimmun Rev. 12 (12): 1129–35. doi:10.1016/j.autrev.2013.06.016. PMC 4086751. PMID 23820041.
  5. ↑ Orriols R, Muñoz X, Ferrer J, Huget P, Morell F (January 1996). “Cocaine-induced Churg-Strauss vasculitis”. Eur. Respir. J. 9 (1): 175–7. PMID 8834352.
  6. ↑ Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM (September 2007). “HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome”. Arthritis Rheum. 56 (9): 3159–66. doi:10.1002/art.22834. PMID 17763415.
  7. ↑ Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT (June 2008). “Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener’s granulomatosis”. Arthritis Rheum. 58 (6): 1839–48. doi:10.1002/art.23496. PMID 18512809.

Template:WH Template:WS

Differentiating Churg-Strauss syndrome from other Diseases

Differentiating Churg-Strauss syndrome from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]Eiman Ghaffarpasand, M.D. [4]

Overview

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that can cause eosinophilia, purpura, alveolar hemorrhage, necrotizing extra-capillary glomerulonephritis, such as granulomatosis with polyangiitis and microscopic polyangiitis.

Differentiating Eosinophilic granulomatosis with polyangiitis from other Diseases

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that cause purpura, alveolar hemorrhage, necrotizing extra-capillary glomerulonephritis, such as granulomatosis with polyangiitis and Microscopic polyangiitis.[1]

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis serological findings
Eosinophilic granulomatosis with polyangiitis Granulomatosis with polyangiitis Microscopic polyangiitis
Cytoplasmic ANCA (cANCA) ≠ 90% positive ≠
Perinuclear ANCA (pANCA) 30 to 40% positive ≠ 60 to 80% positive
Myeloperoxidase antigen 40% sensitivity 10% sensitivity 30% sensitivity
Proteinase 3 antigen <5% sensitivity 70-80% sensitivity 60% sensitivity

Differentiating Eosinophilic granulomatosis with polyangiitis from other Diseases

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that cause pulmonary eosinophilia and perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) such as:[2]

Pulmonary eosinophilia

Perinuclear ANCA


Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other
Large-Vessel Vasculitis Takayasu arteritis[3] + +/- + – + + +/- +/- MMP-3 and MMP-9 Leukocytosis, Anemia ↑ ↑CRP Aneurysmal dilatation of the aorta Blood vessel stenosis Circumferential thickening of the arterial wall (Macaroni sign) PET-scan, Cardiac CT Granulomatous inflammation of arteries Arteriography Coronary aneurysm
Giant cell arteritis[4] + – + +/- – – – +/- Pentraxin 3 (PTX3) Normal ↑ ↑CRP Stenosis, Occlusion, Dilatation Aneurysm – Mural inflammation in MRA Granulomatous inflammation of arteries Biopsy  Jaw pain and claudication
Neurological disease Cerebral aneurysm[5] + – – – – – +/- + – Normal Normal – Well-defined round, slightly hyperattenuating lesion Bulging out of the main lumen – Heterogeneous signal intensity in MRA Layers of smooth muscle cells, Intact endothelium Digital subtraction angiography (DSA) Nausea, Vomiting
Neurofibromatosis type 1[6] +/- – +/- – – – + + NF1 mutated gene Normal Normal Neurofibromin gene Bone abnormalities  Optical coherence tomography angiography (OCTA) – Optic nerve gliomas in MRI Elongated spindle-shaped cells in neurofibromas NIH diagnostic criteria Cafe au Lait spot
Neurofibromatosis type 2[7] +/- – +/- – +/- – – + NF2 mutated gene Normal Normal Schwannomin Meningioma, Schwannoma, Ependymoma Fluorescein angiography showed retinal hamartoma Localized schwannomas in nerve ultrasound Schwannoma in MRI Encapsulated biphasic nerve sheath tumor NIH diagnostic criteria Hearing loss, Vision loss
Systemic disease Fibromuscular dysplasia[8] + – +/- + + + + +/- Transforming growth factor β (TGF-β) Normal ↑ ↑ Cr or BUN Alternating stenosis and dilatations in CT angiography Stenosis in the renal arteries Luminal narrowing alternating with dilatation (Beads sign) Focal concentric, long-segment tubular stenosis or outpouching in MRA Fibrodysplastic changes, Collagen deposition Digital subtraction angiography (DSA)  Spontaneous coronary artery dissection (SCAD)
Ehlers-Danlos syndrome[9] – – – + +/- +/- – +/- TGF-β Normal Normal Cultured skin fibroblasts Multiple vascular segments with aneurysms and dissections Dissection of the posterolateral branch of the left circumflex coronary artery (LCx) Visceral arteries abnormality Vascular abnormalities in MRA Thin and rare collagen bundles in the dermis History and physical examination Bleeding, Bruisability, Heart murmur
Polymyalgia rheumatica (PMR)[10] + – +/- + – – – – Plasma fibrinogen Normocytic, normochromic anemia ↑ CRP Periodontoid localization of calcification Vessel wall thickening, Increased mural contrast enhancement Subacromial or subdeltoid bursitis High F-FDG accumulation around the joints in FDG PET-CT Small angular fibers, Pyknotic nuclear clumps, or target-targetoid fibers – Joint stiffness, Fatigue
Amyloidosis[11] +/- – – +/- +/- – + + Immunoglobulin light chain(Amyloid) Anemia Normal ↑ Cr or BUN,

↑ ALT or AST

Diffusely hypoattenuating and enlarged liver Amyloid deposition in the media and adventitia of small arteries  Solid organs increased echogenicity Tc-DPD for cardiac amyloid deposits Extracellular deposition of fibrillar proteins Biopsy Cardiomegaly, Dyspnea
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other Histopathology Gold standard Additional findings
Medium-Vessel Vasculitis Polyarteritis nodosa[12] + + + + + + +/- +/- LAMP-2 protein autoantibodies Leukocytosis, Normochromic anemia, Thrombocytosis ↑ ↑ Cr or BUN,

↑ ALT or AST, Proteinuria

Focal regions of infarction or hemorrhage Multiple microaneurysms, Hemorrhage due to focal rupture, Occlusion Aneurysms and renal arteriovenous fistula in color Doppler sonography – Necrotizing inflammatory lesions Angiography Sudden weight loss, Abdominal pain
Hepatitis B virus-associated polyarteritis nodosa[13] +/- +/- + + +/- +/- + – HBsAg Leukocytosis, Normochromic anemia, Thrombocytosis ↑ ↑ ALT or AST Focal regions of infarction or hemorrhage Microaneurysms in mesenteric artery Aneurysms and renal arteriovenous fistula in color Doppler sonography – Necrotizing inflammatory lesions Angiography Peripheral neuropathy, Livedo reticularis
Kawasaki disease[14] – + +/- + + – +/- – NT-proBNP, Meprin A, Filamin C Normochromic anemia, ↑WBC with a left shift, Thrombocytosis  ↑ Acute-phase reactants, ↓Cholesterol, ↓HDL, ↓ApolipoA Coronary artery calcifications Coronary artery aneurysms, stenosis or occlusion Coronary artery anomaly in echocardiography Electron beam CT (EBCT) Acute destruction of the media by neutrophils, with loss of elastic fibers History and physical examination Diarrhea, Vomiting
Infectious disease Parvovirus B19 infection[15] + + + + – – – +/- B19 DNA, ↓Reticulocyte count Anemia ↑ anti–parvovirus B19 IgM – – Hydrops in fetal ultrasonography – – B19 DNA Purpuric rash, Erythema multiforme
Scarlet fever[16] + + +/- + – – – – Antistreptolysin-O (ASO) titers Leukocytosis ↑ ↑CRP Thickened pulmonary markings if pneumonia – – – Sparse neutrophilic perivascular infiltrate History and physical examination Sand-paper rashes, Sore throat
Toxic shock syndrome[17] + + + + – – – +/- Procalcitonin Leukocytosis with left shift ↑ Myoglobinuria, Sterile pyuria Acute respiratory distress syndrome – – – Necrolysis of keratinocytes in epidermis, Perivascular lymphocytic infiltrate Clinical criteria Peeling or rashes, Organ dysfunction
Mononucleosis[18] + + + + – – – – EBV DNA Atypical lymphocyte ↑ Heterophile antibodies CNS involvement – Splenomegaly Encephalitis in MRI Lymphoproliferative response in oropharynx, Lymphocytic infiltration in spleen Heterophile antibody test Splenomegaly, Palatal petechiae
Leptospirosis[19] + + + + +/- – – – IL-6, IL-8 and IL-10 Anemia – ↑Cr or BUN,

↑ALT or AST, Proteinuria

 Diffuse alveolar hemorrhage – – – Toxin-mediated break down of endothelial cell membranes of capillaries Culture and the microscopic agglutination test Red eyes, Skin rash
Lyme Disease[20] +/- + +/- + +/- – – – CXCL9 (MIG), CXCL10 (IP-10) and CCL19 (MIP3B) Leukopenia, Thrombocytopenia – Microscopic hematuria, Proteinuria, ↑ALT or AST Punctate lesions in periventricular white matter in brain SPECT – – – Acrodermatitis chronica atrophicans Serologic tests Erythema migrans
Measles[21] +/- + +/- + – – – – Measles IgM Leukopenia, Lymphocytosis, Thrombocytopenia – ↑ALT or AST Pneumonia – – CXR Spongiosis and vesiculation in the epidermis with scattered dyskeratotic keratinocytes PCR Generalized rash, Cough, Coryza, or Conjunctivitis
Rocky Mountain Spotted Fever[22] + + + + – – – – R rickettsii serology Thrombocytopenia, Anemia  – ↑ALT or AST, Hyponatremia Infarction, edema, and meningeal enhancement – Myocardial or conduction abnormalities in echocardiography – Immunofluorescent or immunoperoxidase staining of R rickettsii Clinical criteria and tick exposure Rash on the palms and soles
Staphylococcal Scalded Skin Syndrome[23] + + + + – – +/- +/- Anti exfoliatin and anti alpha-toxin antibodies Leukocytosis with left shift ↑ Blood culture Pneumonia – – – Intraepidermal blister, dense superficial perivascular lymphohistiocytic infiltrate  Blood culture and clinical findings Widespread skin erythema, fluid-filled blisters
Toxic Epidermal Necrolysis[24] – + + – – – – +/- MicroRNA-124 Normochromic normocytic anemia, Eosinophilia ↑ Fluid loss and electrolyte abnormalities Tracheobronchial inflammation – – – Necrotic keratinocytes with full-thickness epithelial necrosis Histopathology and clinical findings Erythematous macular rash with purpuric centers
Cardiovascular disease Atrial Myxoma[25] – – +/- – – – – +/- Calretinin Mild anemia, Leukocytosis ↑ ↑IL-6 Atrial filling defect larger than a thrombus – Tumor location, size, attachment, and mobility in echocardiography Size, shape, and surface characteristics in MRI Lipidic cells embedded in a vascular myxoid stroma Echocardiography Dyspnea on exertion, Syncope
Cholesterol Embolism[26] +/- +/- – – + – – + IL-5 Eosinophilia, Leukocytosis   ↑ Eosinophiluria Thoracic and abdominal aortic sources of embolism Atheroembolism in abdominal aorta and the lower extremity arteries Excluding an intracardiac source of embolism with echocardiography  – Birefringent crystals or biconvex needle-shaped ghostly clefts within the arterial lumen Angiography  Livedo reticularis,

Ischemic patches

Segmental arterial mediolysis[27] + – – – + + + +/- – Leukocytosis – – Visceral artery aneurysm in CT angiography Alternating aneurysms and stenoses (beading) Retroperitoneal hematoma – Disruption of the smooth muscle in the media Angiography  Hematuria, Ischemic colitis
Systemic disease Antiphospholipid Syndrome[28] + + – – – – – +/- Antiphospholipid antibodies Thrombocytopenia, Hemolytic anemia – Lupus anticoagulant (LA) Stroke,

Pulmonary embolism, Budd-Chiari syndrome

Thrombus in major vessels Valve thickening, vegetations, or insufficiency in echocardiography – Noninflammatory bland thrombosis without perivascular inflammation Hx of thrombosis and antiphospholipid antibodies Miscarriage, Pulmonary hypertension
Juvenile Idiopathic Arthritis[29] – – – + +/- – – – Rheumatoid factor (RF), S100A12 Lymphocytosis, Thrombocytopenia ↑ Myeloid-related proteins 8/14 (MRP8/14) Synovial hypertrophy, Joint effusions Cerebral vasculitis Inflamed synovium Bone scanning Vascular congestion, RBC extravasation, Venular lumen occlusion Conventional radiography Evanescent rash, Dactylitis 
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other Histopathology Gold standard Additional findings
Small-Vessel Vasculitis ANCA-associated vasculitis Microscopic polyangiitis[30] + – – +/- – – – + Anti-PR3 antibody (C-ANCA) (40%), Anti-MPO antibody (P-ANCA) (60%) Leukocytosis, Normocytic anemia ↑ Proteinuria, Erythrocyte casts Suspected pancreatitis Mesenteric angiography for differentiating from polyarteritis nodosa – Bilateral nodular, and patchy opacities in CXR Glomerulonephritis with focal necrosis, crescent formation, and lack or paucity of immunoglobulin deposits Histological confirmation Rash, Hemoptysis 
Granulomatosis with polyangiitis (Wegener’s)[31] + +/- +/- – – – – + Anti-PR3 antibody (C-ANCA) (90%), Anti-MPO antibody (P-ANCA) (10%) Leukocytosis, Normochromic normocytic anemia ↑ ↑Cr or BUN, Hypoalbuminemia Consolidation, Patchy or diffuse ground-glass opacities Occlusion or stenosis of LAD and RCA in coronary angiography – Single or multiple nodules and masses with cavitation in CXR Parenchymal necrosis, Granulomatous inflammation Histological confirmation Conjunctivitis,

Episcleritis,

Uveitis,

Optic nerve vasculitis

Eosinophilic granulomatosis with polyangiitis

(Churg-Strauss)[32]

+/- – – + – – – + Anti-MPO antibody (P-ANCA) (40%), Eotaxin-3 Eosinophilia, Anemia ↑ ↑Cr or BUN, Proteinuria, Erythrocyte casts, ↑IgE levels Significant enlargement of peripheral pulmonary arteries Myocardial ischemia and infarction in coronary angiography Congestive heart failure (CHF) in echocardiography Extensive air-space opacities in CXR Small necrotizing granulomas with eosinophilic core surrounded by macrophages and epithelioid giant cells Histological confirmation Allergic rhinitis, Asthma, Urticarial rash
Hydralazine-associated ANCA-associated vasculitis[33] – – – + – – – +/- Anti-MPO antibody (P-ANCA), Anti-histone antibodies Anemia – ↑Cr or BUN, Hypoalbuminemia Bilateral pulmonary infiltrates Aneurysms or occlusions of the visceral arteries – – Pauci-immune necrotizing and crescentic glomerulonephritis Histological confirmation Sinusitis, Hemoptysis
Immune complex small-vessel vasculitis Anti-glomerular basement membrane disease[34] + +/- – – – – + – Anti-GBM antibodies Hypochromic microcytic anemia, Thrombocytopenia – ↓C3 level Pulmonary hemorrhage – Normal kidneys Alveolar infiltrates spreading from the hilum in CXR Cellular crescents in the glomeruli, Intra-alveolar hemorrhages Anti-GBM antibodies Hemoptysis, Hematuria
Cryoglobulinemic vasculitis[35] +/- +/- – + +/- – – – C4 component Leukocytosis, Anemia ↑ ANA, hypocomplementemia R/O underlying malignancy Stenosis or occlusions of the visceral arteries Bacterial endocarditis in echocardiography Interstitial involvement or pleural effusions in CXR HCV-associated proteins in vasculitic skin, Intraluminal cryoglobulin deposits  Histological confirmation Acrocyanosis, Retinal hemorrhage, Purpura
Hepatitis C virus-associated cryoglobulinemic vasculitis[36] +/- +/- + + – – + +/- HCV RNA, Cryoglobulins Leukocytosis, Anemia ↑ ↓Serum C4, Positive RF Increased hepatic echogenicity – Hepatomegaly, Splenomegaly Increased hepatic echogenicity in MRI Vasculitic skin, Antigen infilteration in lesions HCV RNA, Histological confirmation Palpable purpura, Microscopic hematuria
IgA vasculitis (Henoch-Schönlein purpura)[37] – – + + – – – – IgA Normochromic anemia, Leukocytosis  ↑ Stool OB, ↓C3, ↓C4 – – Increased bowel wall thickness, hematomas, peritoneal fluid, and intussusception Dilated loops of bowel consistent in abdominal X-ray Leukocytoclastic vasculitis in postcapillary venules with IgA deposition History and physical examination Hematuria, Palpable purpura
Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)[38] – – +/- + – – – – C1q Mild anemia ↑ ANA, ↓C1q, ↓C3, ↓C4 – – Hepatomegaly, Splenomegaly – Deposits of immunoglobulins, complement, or fibrin around blood vessels Urticaria,

Histological confirmation

Urticaria, Hematuria
Gastrointestinal disease Acute mesenteric ischemia[39] – +/- – – – – – – I-FABP, Alpha-GST, Ischemia-modified albumin (IMA) Leukocytosis, ↑HCT –  ↑Amylase Bowel wall thickening, Intestinal pneumatosis, Portomesenteric thrombosis Mesenteric venous thrombosis  Arterial stenosis or occlusion of the celiac or superior mesenteric arteries in duplex ultrasound Ileus with distended loops of bowel, Bowel wall thickening in abdominal X-ray Superficial mucosal hemorrhage, edema and necrosis History and physical examination Abdominal pain, Distension, Absent bowel sounds
Cardiovascular disease Infective Endocarditis[40] + + + – – – – + NT-proBNP Normochromic-normocytic anemia ↑ Hyperglobulinemia, Cryoglobulinemia Metastatic infections, such as splenic infarct, renal infarcts, or psoas abscess – Vegetation, abscess, or new dehiscence of a prosthetic valvein echocardiography Vertebral osteomyelitis in MRI Vegetation or intracardiac abscess demonstrating active endocarditis Echocardiography (TTE) Janeway lesions, Osler nodes, Roth spots, Vertebral osteomyelitis
Leukocytoclastic Vasculitis[41] + – – + – – – + IgM, IgA, IgG Leukocytosis, Anemia ↑ Hypocomplementemia – Vascular stenosis and obstruction in visceral angiography – – Perivascular inflammatory infiltrate of neutrophils with leukocytoclasia (releasing nuclear debris) Histological confirmation Palpable purpura, Petechiae 
Pulmonary disease Langerhans Cell Histiocytosis[42] +/- – – + – – – – CD1a, CD207,BRAF-V600E Anemia ↑ Hypercalcemia Pulmonary cysts and nodules, Bone lytic lesions – Hepatomegaly, Splenomegaly Cerebellum and pons hyperintensity in MRI Birbeck granules by electron microscopy Histological confirmation Brown to purplish papules, Eczematous rash
Non-Small Cell Lung Cancer[43] – +/- + – – – – +/- EGFR, ROS1, EML4-ALK,  PD-L1 Leukocytosis, Anemia ↑ Hypercalcemia, Hyponatremia Pulmonary lesion or mass – Pulmonary marginal lesions Staging and response to treatment in PET-CT Adenocarcinoma, Squamous cell carcinoma High resolution CT-scan Cough, Hemoptysis
Small Cell Lung Cancer[44] – +/- + – – – – +/- p53, Thyroid transcription factor-1 (TTF1) Anemia ↑ Hyponatremia Large hilar mass with bulky mediastinal adenopathy – Endobronchial ultrasound (EBUS) Standard staging Spindled cells with dark nuclei, scant cytoplasm, and fine, granular nuclear chromatin High resolution CT-scan Cough, Hemoptysis
Pulmonary Infarction[45] +/- – – – – – + – D-dimer Mild leukocytosis, Mild anemia – Hypoxemia, Hypocarbia or Hypercarbia, Respiratory alkalosis Pulmonary embolism Low-density filling defect within the pulmonary artery Pericardial effusion in echocardiography Pulmonary infiltrates, atelectasis, and pleural effusions in CXR Infarct induced apoptosis Pulmonary artery angiography Cough, Hemoptysis
Renal disease Acute Poststreptococcal Glomerulonephritis[46] – – – + – – + +/- Antistreptolysin-O (ASO) titers Leukocytosis ↑ Hypocomplementemia – – Normal to slightly enlarged kidneys Central venous congestion in a hilar pattern in CXR Hypercellularity of endothelial and mesangial cells, Infiltration of the glomerular tuft with polymorphonuclear cells Histological confirmation Hematuria
Hematologic disease Hemolytic-Uremic Syndrome[47] + + – + – – + + C5b-9, ADAMTS13 Anemia, Thrombocytopenia, Reticulocytosis  ↑ ↑Lactate dehydrogenase (LDH), Hypercalcemia  Thalami, brainstem, or cerebellum abnormality Cerebral microangiopathy or hypertension Hypoechoic kidney  Abnormal hyperintensity in the brain cisterns in MRI Microthromboses include fibrin thrombi that may occlude the glomerular tuft Clinical findings coupled with laboratory abnormalities Hematuria, Proteinuria 
Chronic Lymphocytic Leukemia (CLL)[48] + + + + +/- – – +/- CD5, CD19, CD20, IgVH Absolute lymphocytosis, Smudge cells ↑ Flow cytometry Staging – – – Large atypical cells, cleaved cells, and prolymphocytes  Chromosomal and genetic testing Easy bruising
Multiple Myeloma[49] + – + + + + +/- +/- Ig light chain Anemia, Thrombocytopenia, Leukopenia ↑ Bone marrow aspiration and biopsy, ↑Cr Osseous involvement and lytic lesions Peripheral zone of increased vascularity in lesions – Punched-out lesion in skull X-ray Clonal proliferation of plasma cells Protein electrophoresis plus conventional X-rays Constipation
Hypereosinophilic Syndrome[50] +/- +/- – – – – – – IgE, CD117 with CD2 Eosinophilia – ↑Serum tryptase Lymphadenopathy and splenomegaly – Intracardiac thrombi in echocardiography – Reticulin stain for myelofibrosis and tryptase staining for mast cells Clinical findings coupled with laboratory abnormalities Splinter hemorrhages, Raynaud phenomenon
Non-Hodgkin Lymphoma[51] + + + + +/- +/- +/- +/- MYC, BCL2, BCL6, and TP53 Lymphocytosis, Anemia, Thrombocytopenia ↑ ↑Lactate dehydrogenase (LDH), Hypercalcemia  Enlarged lymph nodes, Hepatosplenomegaly, Filling defects in the liver and spleen – Hepatosplenomegaly Mediastinal lymphadenopathy Small cleaved or noncleaved, intermediate, or large cell with a follicular or diffuse pattern Surgically excised tissue biopsy Easy bruising, Testicular mass, Skin lesion
Serum Sickness[52] + + – +/- +/- – – +/- IL-1, IL-6, TNF Leukopenia  ↑ Polyclonal gammopathy, ↑Cr, Cryoglobulinemia – – – – Arteritic lesions are focal, necrotizing, and inflammatory involving all layers of the artery Clinical findings coupled with laboratory abnormalities Hematuria, Skin rash
Disseminated Intravascular Coagulation[53] +/- + – +/- – – + + Fibrin degradation product (FDP) Thrombocytopenia, Schistocytes ↑ ↑D-dimer, aPTT and PT Intracranial hemorrhage – – – Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels Clinical findings coupled with laboratory abnormalities  Acral cyanosis, Hemorrhagic skin infarctions
Idiopathic Thrombocytopenic Purpura[54] + +/- – + – – – + FC gamma receptors (FCGR) IIb Anemia, Thrombocytopenia – HIV, ANA R/O other causes – R/O splenomegaly – Increased number of normal morphologic megakaryocytes Clinical findings coupled with thrombocytopenia Easy bruising, Purpura
Systemic disease Sarcoidosis[55] + + + + – – – +/-  IL-2 and IFN-γ Mild anemia ↑ ↑ACE, ↑1, 25-dihydroxyvitamin D Active alveolitis or fibrosis – Hepatosplenomegaly Bilateral hilar adenopathy Noncaseating granulomas (NCGs) Histological confirmation Heart block, Ocular lesion
Legionella Infection[56] + + + + – – – +/- Inflammatory cytokines Leukocytosis with left shift, Thrombocytosis ↑ ↑D-dimer, FDP, Hyponatremia Pleural effusion – – Nonspecific and indistinguishable CXR Intra-alveolar inflammation, Microabscesses in the parenchyma Sputum culture Cough, Diarrhea
Systemic lupus erythematosus[57] + + + + – – + + Anti dsDNA, ANA  Leukopenia, Lymphopenia, Anemia, Thrombocytopenia ↑ ↑Cr or BUN,

↑ALT or AST, Proteinuria

Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar hemorrhage Aneurysms Pericardial effusion, pulmonary hypertension, or verrucous Libman-Sacks endocarditis in echocardiography Central nervous system (CNS) lupus white-matter changes in MRI Staging lupus nephritis Anti-dsDNA antibody test Skin rashes or photosensitivity
Rheumatoid arthritis[58] – – + + + + – – RF, Anti-CCP antibody Anemia ↑ ↑Cr or BUN,

↑ALT or AST, ANA

Microfractures Aneurysms Effusions in joints Basilar invagination with cranial migration of an eroded odontoid peg in MRI Influx of inflammatory cells into the synovial membrane, with angiogenesis, proliferation of chronic inflammatory cells Clinical findings coupled anti-CCP antibody Rheumatoid nodules
Relapsing polychondritis[59] – +/- +/- + + – – – – Leukocytosis, Anemia – Cryoglobulins, ANA, C-ANCA Calcification of cartilaginous structures Aortic root dilatation Aortic root dilatation and degree of aortic regurgitation in echocardiography Tracheal stenosis in CXR Chondrolysis, Chondritis, Perichondritis Clinical findings coupled with imaging Ear pain and redness, Polyarthritis
Diseases Headache Fever Weight loss Arthralgia Claudication Bruit HTN Focal neurological disorder Biomarker CBC ESR Other CT scan Angiography Ultrasound/ Echocardiography Other Histopathology Gold standard Additional findings
Variable-vessel vasculitis Behçet’s syndrome[60] – – – + – – +/- – CXCL1  Mild anemia, Neutrophilia ↑ Factor V Leiden Focal CNS lesions Aneurysm formation and thrombosis areas Valve vegetations and ventricular thrombi in echocardiography Meningoencephalitis  in MRI Lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis Clinical criteria Genital ulcerations, Oral ulceration
Cogan’s syndrome[61] +/- – – +/- – – – +  Anti-Hsp70 antibodies Anemia, Thrombocytosis ↑ RF, ANA Thickening and enhancement of both posterior sclera  Stenosis, thrombosis or more lesions in aortic root Aortic insufficiency in echocardiography Early interstitial keratitis by slit lamp Muscle necrosis and atrophy resembling myositis – Red eye, Hearing loss, Vertigo
Gastrointestinal disease Inflammatory Bowel Disease[62] +/- + + + + – – – Anti-Saccharomyces cerevisiae antibody (ASCA), P-ANCA Leukocytosis, Anemia ↑  Iron or vitamin deficiency, Stool OB Mesenteric fat stranding, bowel wall enhancement, increased vascularity (comb sign) – Fistulas, Abscesses, Stenosis Grossly denuded mucosa with active bleeding in colonoscopy Crypt abscesses and mucosal ulceration, Granulomas  Endoscopy  GI bleeding
Whipple’s disease[63] + +/- + + + + + + CCR6, Gut-homing marker integrin β7-chain, T whippelii DNA Mild anemia, Neutrophilia ↑ 72-hour fecal fat determination Nonspecific malabsorption – Hepatosplenomegaly – Periodic acid-Schiff–positive macrophages infiltration in lamina propria of the small bowel Broad-spectrum PCR amplifications Cachexia,

Glossitis

Sjögren’s syndrome[64] – – +/- + – – – – Anti-Ro and Anti-La, Anti-alpha-fodrin antibody Anemia,

Leukopenia,

Eosinophilia

↑ Hypergammaglobulinemia, Low bicarbonate level, Hypokalemia  Salt and pepper or honeycomb appearance in parotid glands – Multicystic or reticular pattern in atrophic salivary gland R/O obstructions or strictures with Sialography  Focal aggregates of lymphocytes Schirmer test Keratoconjunctivitis, Gingival inflammation
Single-organ vasculitis Primary central nervous system vasculitis[65] + + + – + – + + von Willebrand factor antigen (vWF) Normal – CSF pleocytosis, predominantly lymphocytes Cerebral infarcts or hemorrhages with mass effect, or hydrocephalus Aneurysm in circle of Willis – Progression of the disease or response to therapy in MRI Chronic granulomatous inflammation and giant cells Histological confirmation Skin rash, Purpura
Infectious disease Aspergillosis[66] + + + – – – – + Aspergillus nucleic acid in blood, Galactomannan Eosinophilia ↑ ↑Serum IgE Aspergilloma mass within a cavity Mass effect stenosis – Aspergilloma mass within the brain in MRI Septate hyphae, branching at acute angles, and tissue necrosis with granulomata and blood vessel invasion Histological confirmation Hemoptysis, Aspergilloma
Histoplasmosis[67] + + + + + – – + – Mild anemia ↑ ↑ALP, ↑LDH Cerebral histoplasmosis  – Valvular involvement in echocardiography PFT Presence of yeast forms in tissue through hematoxylin and eosin staining Sputum cultures Pneumonia, Mediastinitis
Herpes Simplex Encephalitis[68] + + – – – – – + HSV DNA Mild lymphocytosis ↑ CSF pleocytosis Low-density lesions in the temporal and/or frontal lobe – – Hemorrhagic lesion in white matter Multinuclear giant cells PCR or brain biopsy Seizures,

Vomiting

Systemic disease Eclampsia[69] + + – + – – + + VEGF, PlGF, Soluble FLT-1 Anemia, Thrombocytopenia, Schistocytes – ↑Bilirubin, ↓Haptoglobin, ↑LDH, ↑Cr Cortical hypodense areas in the occipital lobes, Diffuse cerebral edema – Poor fetal growth, Oligohydramnios, Abnormal umbilical artery  Increased signal at the gray-white matter junction in MRI – 24-hour urine study  Seizure, Edema
Fibromuscular dysplasia[8] + – +/- + + + + +/- Transforming growth factor β (TGF-β) Normal ↑ ↑ Cr or BUN Alternating stenosis and dilatations in CT angiography Stenosis in the renal arteries Luminal narrowing alternating with dilatation (Beads sign) Focal concentric, long-segment tubular stenosis or outpouching in MRA Fibrodysplastic changes, Collagen deposition Digital subtraction angiography (DSA)  Spontaneous coronary artery dissection (SCAD)

Diagnosis of Eosinophilic granulomatosis with polyangiitis

In order to make a diagnosis of Eosinophilic granulomatosis with polyangiitis the following criteria must be present:

According to the American College of Rheumatology classification criteria [70]

Asthma

Eosinophilia

Polyneuropathy or Mononeuropathy

Non fixed pulmonary infiltrates

Paranasal sinus that is abnormal

Eosinophils that are extravascular

Patients must express 4 out the 6 criteria to be diagnosed with eosinophilic granulomatosis with polyangiitis.

According to Lanham diagnostic criteria [71]

Asthma

Eosinophilia peak of >1.5×109 cell/L or >10% of the total WBC

Systemic vasculitis, two or greater extra pulmonary sites

All 3 criteria’s need to be present

References

  1. ↑ Pagnoux C (2016). “Updates in ANCA-associated vasculitis”. Eur J Rheumatol. 3 (3): 122–133. doi:10.5152/eurjrheum.2015.0043. PMID 27733943.
  2. ↑ Conron M, Beynon HL (2000). “Churg-Strauss syndrome”. Thorax. 55 (10): 870–7. PMC 1745623. PMID 10992542.
  3. ↑ Vaideeswar P, Deshpande JR (2013). “Pathology of Takayasu arteritis: A brief review”. Ann Pediatr Cardiol. 6 (1): 52–8. doi:10.4103/0974-2069.107235. PMC 3634248. PMID 23626437.
  4. ↑ Calvo-Romero JM (2003). “Giant cell arteritis”. Postgrad Med J. 79 (935): 511–5. PMC 1742823. PMID 13679546.
  5. ↑ Stafa A, Leonardi M (2008). “Role of neuroradiology in evaluating cerebral aneurysms”. Interv Neuroradiol. 14 Suppl 1: 23–37. doi:10.1177/15910199080140S106. PMC 3328052. PMID 20557771.
  6. ↑ Cassiman C, Casteels I, Stalmans P, Legius E, Jacob J (2017). “Optical Coherence Tomography Angiography of Retinal Microvascular Changes Overlying Choroidal Nodules in Neurofibromatosis Type 1”. Case Rep Ophthalmol. 8 (1): 214–220. doi:10.1159/000469702. PMC 5422752. PMID 28512424.
  7. ↑ Evans, D G. R (2000). “Neurofibromatosis type 2”. Journal of Medical Genetics. 37 (12): 897–904. doi:10.1136/jmg.37.12.897. ISSN 1468-6244.
  8. ↑ 8.0 8.1 Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X (2007). “Fibromuscular dysplasia”. Orphanet J Rare Dis. 2: 28. doi:10.1186/1750-1172-2-28. PMC 1899482. PMID 17555581.
  9. ↑ Gazit Y, Jacob G, Grahame R (2016). “Ehlers-Danlos Syndrome-Hypermobility Type: A Much Neglected Multisystemic Disorder”. Rambam Maimonides Med J. 7 (4). doi:10.5041/RMMJ.10261. PMC 5101008. PMID 27824552.
  10. ↑ Michet CJ, Matteson EL (2008). “Polymyalgia rheumatica”. BMJ. 336 (7647): 765–9. doi:10.1136/bmj.39514.653588.80. PMC 2287267. PMID 18390527.
  11. ↑ Baker KR, Rice L (2012). “The amyloidoses: clinical features, diagnosis and treatment”. Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
  12. ↑ Howard T, Ahmad K, Swanson JA, Misra S (2014). “Polyarteritis nodosa”. Tech Vasc Interv Radiol. 17 (4): 247–51. doi:10.1053/j.tvir.2014.11.005. PMC 4363102. PMID 25770638.
  13. ↑ Sharma A, Sharma K (September 2013). “Hepatotropic viral infection associated systemic vasculitides-hepatitis B virus associated polyarteritis nodosa and hepatitis C virus associated cryoglobulinemic vasculitis”. J Clin Exp Hepatol. 3 (3): 204–12. doi:10.1016/j.jceh.2013.06.001. PMC 4216827. PMID 25755502.
  14. ↑ Takahashi K, Oharaseki T, Yokouchi Y (2011). “Pathogenesis of Kawasaki disease”. Clin Exp Immunol. 164 Suppl 1: 20–2. doi:10.1111/j.1365-2249.2011.04361.x. PMC 3095860. PMID 21447126.
  15. ↑ Heegaard ED, Brown KE (2002). “Human parvovirus B19”. Clin Microbiol Rev. 15 (3): 485–505. PMC 118081. PMID 12097253.
  16. ↑ Basetti S, Hodgson J, Rawson TM, Majeed A (2017). “Scarlet fever: a guide for general practitioners”. London J Prim Care (Abingdon). 9 (5): 77–79. doi:10.1080/17571472.2017.1365677. PMC 5649319. PMID 29081840.
  17. ↑ Vostral SL (2011). “Rely and Toxic Shock Syndrome: a technological health crisis”. Yale J Biol Med. 84 (4): 447–59. PMC 3238331. PMID 22180682.
  18. ↑ Balfour HH, Dunmire SK, Hogquist KA (2015). “Infectious mononucleosis”. Clin Transl Immunology. 4 (2): e33. doi:10.1038/cti.2015.1. PMC 4346501. PMID 25774295.
  19. ↑ Levett PN (April 2001). “Leptospirosis”. Clin. Microbiol. Rev. 14 (2): 296–326. doi:10.1128/CMR.14.2.296-326.2001. PMC 88975. PMID 11292640.
  20. ↑ Biesiada G, Czepiel J, Leśniak MR, Garlicki A, Mach T (2012). “Lyme disease: review”. Arch Med Sci. 8 (6): 978–82. doi:10.5114/aoms.2012.30948. PMC 3542482. PMID 23319969.
  21. ↑ White SJ, Boldt KL, Holditch SJ, Poland GA, Jacobson RM (2012). “Measles, mumps, and rubella”. Clin Obstet Gynecol. 55 (2): 550–9. doi:10.1097/GRF.0b013e31824df256. PMC 3334858. PMID 22510638.
  22. ↑ Walker DH (1989). “Rocky Mountain spotted fever: a disease in need of microbiological concern”. Clin Microbiol Rev. 2 (3): 227–40. PMC 358117. PMID 2504480.
  23. ↑ Mishra AK, Yadav P, Mishra A (2016). “A Systemic Review on Staphylococcal Scalded Skin Syndrome (SSSS): A Rare and Critical Disease of Neonates”. Open Microbiol J. 10: 150–9. doi:10.2174/1874285801610010150. PMC 5012080. PMID 27651848.
  24. ↑ Hoetzenecker W, Mehra T, Saulite I, Glatz M, Schmid-Grendelmeier P, Guenova E; et al. (2016). “Toxic epidermal necrolysis”. F1000Res. 5. doi:10.12688/f1000research.7574.1. PMC 4879934. PMID 27239294.
  25. ↑ MacGowan SW, Sidhu P, Aherne T, Luke D, Wood AE, Neligan MC, McGovern E (June 1993). “Atrial myxoma: national incidence, diagnosis and surgical management”. Ir J Med Sci. 162 (6): 223–6. PMID 8407260.
  26. ↑ Avci G, Akoz T, Gul AE (2009). “Cutaneous cholesterol embolization”. J Dermatol Case Rep. 3 (2): 27–9. doi:10.3315/jdcr.2009.1031. PMC 3157794. PMID 21886725.
  27. ↑ Chao, Christine (2009). “Segmental Arterial Mediolysis”. Seminars in Interventional Radiology. 26 (03): 224–232. doi:10.1055/s-0029-1225666. ISSN 0739-9529.
  28. ↑ Chaturvedi S, McCrae KR (2015). “The antiphospholipid syndrome: still an enigma”. Hematology Am Soc Hematol Educ Program. 2015: 53–60. doi:10.1182/asheducation-2015.1.53. PMC 4877624. PMID 26637701.
  29. ↑ Espinosa M, Gottlieb BS (July 2012). “Juvenile idiopathic arthritis”. Pediatr Rev. 33 (7): 303–13. doi:10.1542/pir.33-7-303. PMID 22753788.
  30. ↑ Chung SA, Seo P (2010). “Microscopic polyangiitis”. Rheum Dis Clin North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
  31. ↑ Kubaisi B, Abu Samra K, Foster CS (2016). “Granulomatosis with polyangiitis (Wegener’s disease): An updated review of ocular disease manifestations”. Intractable Rare Dis Res. 5 (2): 61–9. doi:10.5582/irdr.2016.01014. PMC 4869584. PMID 27195187.
  32. ↑ Keogh KA, Specks U (April 2006). “Churg-Strauss syndrome”. Semin Respir Crit Care Med. 27 (2): 148–57. doi:10.1055/s-2006-939518. PMID 16612766.
  33. ↑ Keasberry J, Frazier J, Isbel NM, Van Eps CL, Oliver K, Mudge DW (2013). “Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis presenting with a vasculitic syndrome, acute nephritis and a puzzling skin rash: a case report”. J Med Case Rep. 7: 20. doi:10.1186/1752-1947-7-20. PMC 3565908. PMID 23316942.
  34. ↑ McAdoo SP, Pusey CD (July 2017). “Anti-Glomerular Basement Membrane Disease”. Clin J Am Soc Nephrol. 12 (7): 1162–1172. doi:10.2215/CJN.01380217. PMID 28515156.
  35. ↑ Ferri C, Mascia MT (January 2006). “Cryoglobulinemic vasculitis”. Curr Opin Rheumatol. 18 (1): 54–63. PMID 16344620.
  36. ↑ Guo QY, Wu M, Wang YW, Sun GD (2017). “Hepatitis C virus-associated cryoglobulinemia with membrano-proliferative glomerulonephritis treated with prednisolone and interferon: A case report”. Exp Ther Med. 14 (2): 1395–1398. doi:10.3892/etm.2017.4671. PMC 5525644. PMID 28810602.
  37. ↑ Farhadian JA, Castilla C, Shvartsbeyn M, Meehan SA, Neimann A, Pomeranz MK (December 2015). “IgA vasculitis (Henoch-Schönlein purpura)”. Dermatol. Online J. 21 (12). PMID 26990342.
  38. ↑ Buck A, Christensen J, McCarty M (2012). “Hypocomplementemic urticarial vasculitis syndrome: a case report and literature review”. J Clin Aesthet Dermatol. 5 (1): 36–46. PMC 3277093. PMID 22328958.
  39. ↑ Sise MJ (February 2014). “Acute mesenteric ischemia”. Surg. Clin. North Am. 94 (1): 165–81. doi:10.1016/j.suc.2013.10.012. PMID 24267504.
  40. ↑ McDonald JR (2009). “Acute infective endocarditis”. Infect Dis Clin North Am. 23 (3): 643–64. doi:10.1016/j.idc.2009.04.013. PMC 2726828. PMID 19665088.
  41. ↑ Einhorn J, Levis JT (2015). “Dermatologic Diagnosis: Leukocytoclastic Vasculitis”. Perm J. 19 (3): 77–8. doi:10.7812/TPP/15-001. PMC 4500485. PMID 26176572.
  42. ↑ Margo CE, Goldman DR (2008). “Langerhans cell histiocytosis”. Surv Ophthalmol. 53 (4): 332–58. doi:10.1016/j.survophthal.2008.04.007. PMID 18572052.
  43. ↑ Molina JR, Yang P, Cassivi SD, Schild SE, Adjei AA (2008). “Non-small cell lung cancer: epidemiology, risk factors, treatment, and survivorship”. Mayo Clin Proc. 83 (5): 584–94. doi:10.4065/83.5.584. PMC 2718421. PMID 18452692.
  44. ↑ Jackman DM, Johnson BE (2005). “Small-cell lung cancer”. Lancet. 366 (9494): 1385–96. doi:10.1016/S0140-6736(05)67569-1. PMID 16226617.
  45. ↑ Parambil JG, Savci CD, Tazelaar HD, Ryu JH (April 2005). “Causes and presenting features of pulmonary infarctions in 43 cases identified by surgical lung biopsy”. Chest. 127 (4): 1178–83. doi:10.1378/chest.127.4.1178. PMID 15821192.
  46. ↑ VanDeVoorde RG (January 2015). “Acute poststreptococcal glomerulonephritis: the most common acute glomerulonephritis”. Pediatr Rev. 36 (1): 3–12, quiz 13. doi:10.1542/pir.36-1-3. PMID 25554106.
  47. ↑ Corrigan JJ, Boineau FG (November 2001). “Hemolytic-uremic syndrome”. Pediatr Rev. 22 (11): 365–9. PMID 11691946.
  48. ↑ Byrd JC, Stilgenbauer S, Flinn IW (2004). “Chronic lymphocytic leukemia”. Hematology Am Soc Hematol Educ Program: 163–83. doi:10.1182/asheducation-2004.1.163. PMID 15561682.
  49. ↑ Michels TC, Petersen KE (March 2017). “Multiple Myeloma: Diagnosis and Treatment”. Am Fam Physician. 95 (6): 373–383. PMID 28318212.
  50. ↑ Klion A (2009). “Hypereosinophilic syndrome: current approach to diagnosis and treatment”. Annu. Rev. Med. 60: 293–306. doi:10.1146/annurev.med.60.062107.090340. PMID 19630574.
  51. ↑ Shankland KR, Armitage JO, Hancock BW (September 2012). “Non-Hodgkin lymphoma”. Lancet. 380 (9844): 848–57. doi:10.1016/S0140-6736(12)60605-9. PMID 22835603.
  52. ↑ Lin RY (January 1986). “Serum sickness syndrome”. Am Fam Physician. 33 (1): 157–62. PMID 2867672.
  53. ↑ Venugopal A (2014). “Disseminated intravascular coagulation”. Indian J Anaesth. 58 (5): 603–8. doi:10.4103/0019-5049.144666. PMC 4260307. PMID 25535423.
  54. ↑ Nomura S (2016). “Advances in Diagnosis and Treatments for Immune Thrombocytopenia”. Clin Med Insights Blood Disord. 9: 15–22. doi:10.4137/CMBD.S39643. PMC 4948655. PMID 27441004.
  55. ↑ Chiarchiaro J, Chen BB, Gibson KF (2016). “New molecular targets for the treatment of sarcoidosis”. Curr Opin Pulm Med. 22 (5): 515–21. doi:10.1097/MCP.0000000000000304. PMC 5152532. PMID 27454074.
  56. ↑ Murdoch DR (January 2003). “Diagnosis of Legionella infection”. Clin. Infect. Dis. 36 (1): 64–9. doi:10.1086/345529. PMID 12491204.
  57. ↑ Tsokos, George C. (2011). “Systemic Lupus Erythematosus”. New England Journal of Medicine. 365 (22): 2110–2121. doi:10.1056/NEJMra1100359. ISSN 0028-4793.
  58. ↑ Scott JT (1991). “The gold standard in rheumatoid arthritis”. J R Soc Med. 84 (9): 513–4. PMC 1293405. PMID 1682491.
  59. ↑ Emmungil H, Aydın SZ (2015). “Relapsing polychondritis”. Eur J Rheumatol. 2 (4): 155–159. doi:10.5152/eurjrheum.2015.0036. PMC 5047229. PMID 27708954.
  60. ↑ Yazici H, Fresko I, Yurdakul S (March 2007). “Behçet’s syndrome: disease manifestations, management, and advances in treatment”. Nat Clin Pract Rheumatol. 3 (3): 148–55. doi:10.1038/ncprheum0436. PMID 17334337.
  61. ↑ Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C (2015). “COGAN’S SYNDROME”. Rom J Ophthalmol. 59 (1): 6–13. PMC 5729811. PMID 27373108.
  62. ↑ Wehkamp J, Götz M, Herrlinger K, Steurer W, Stange EF (2016). “Inflammatory Bowel Disease”. Dtsch Arztebl Int. 113 (5): 72–82. doi:10.3238/arztebl.2016.0072. PMC 4782273. PMID 26900160.
  63. ↑ Dutly F, Altwegg M (2001). “Whipple’s disease and “Tropheryma whippelii““. Clin Microbiol Rev. 14 (3): 561–83. doi:10.1128/CMR.14.3.561-583.2001. PMC 88990. PMID 11432814.
  64. ↑ Stefanski AL, Tomiak C, Pleyer U, Dietrich T, Burmester GR, Dörner T (2017). “The Diagnosis and Treatment of Sjögren’s Syndrome”. Dtsch Arztebl Int. 114 (20): 354–361. doi:10.3238/arztebl.2017.0354. PMC 5471601. PMID 28610655.
  65. ↑ Benseler SM, Silverman E, Aviv RI, Schneider R, Armstrong D, Tyrrell PN, deVeber G (April 2006). “Primary central nervous system vasculitis in children”. Arthritis Rheum. 54 (4): 1291–7. doi:10.1002/art.21766. PMID 16575852.
  66. ↑ Latgé JP (1999). “Aspergillus fumigatus and aspergillosis”. Clin Microbiol Rev. 12 (2): 310–50. PMC 88920. PMID 10194462.
  67. ↑ Guimarães AJ, Nosanchuk JD, Zancopé-Oliveira RM (2006). “DIAGNOSIS OF HISTOPLASMOSIS”. Braz J Microbiol. 37 (1): 1–13. doi:10.1590/S1517-83822006000100001. PMC 2863343. PMID 20445761.
  68. ↑ Sköldenberg B (1996). “Herpes simplex encephalitis”. Scand J Infect Dis Suppl. 100: 8–13. PMID 9163027.
  69. ↑ Uzan J, Carbonnel M, Piconne O, Asmar R, Ayoubi JM (2011). “Pre-eclampsia: pathophysiology, diagnosis, and management”. Vasc Health Risk Manag. 7: 467–74. doi:10.2147/VHRM.S20181. PMC 3148420. PMID 21822394.
  70. ↑ Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP; et al. (1990). “The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis)”. Arthritis Rheum. 33 (8): 1094–100. PMID 2202307.
  71. ↑ Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984). “Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome”. Medicine (Baltimore). 63 (2): 65–81. PMID 6366453.

Template:WH Template:WS

Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]

Overview

The incidence of eosinophilic granulomatosis with polyangiitis ranges from 2.5 to 16.6 per 100,000 individuals. Prevalence ranges from 2 to 16 per 100,000 individuals. Mean age at diagnosis is usually around 45-50 years. In general, eosinophilic granulomatosis with polyangiitis affects men and women equally.

Epidemiology and Demographics

Prevalence

The prevalence of eosinophilic granulomatosis with polyangiitis ranges from 2 to 16 per 100,000 individuals.[1]

Incidence

The incidence of eosinophilic granulomatosis with polyangiitis range from 2.5 to 16.6 per 100,000 individuals.[2]

Demographics

Age

The mean age at diagnosis of eosinophilic granulomatosis with polyangiitis is around 45-50 years.

Gender

In general, there is no gender predilection to eosinophilic granulomatosis with polyangiitis in western nations. In Japan, female predominance has been noted.[3]

Race

In general, there is no racial predominance to eosinophilic granulomatosis with polyangiitis.

References

  1. ↑ Martin RM, Wilton LV, Mann RD (May 1999). “Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies”. Pharmacoepidemiol Drug Saf. 8 (3): 179–89. doi:10.1002/(SICI)1099-1557(199905/06)8:3<179::AID-PDS414>3.0.CO;2-K. PMID 15073927.
  2. ↑ Loughlin JE, Cole JA, Rothman KJ, Johnson ES (March 2002). “Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients”. Ann. Allergy Asthma Immunol. 88 (3): 319–25. doi:10.1016/S1081-1206(10)62015-7. PMID 11926627.
  3. ↑ Sada KE, Amano K, Uehara R, Yamamura M, Arimura Y, Nakamura Y, Makino H (July 2014). “A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan”. Mod Rheumatol. 24 (4): 640–4. doi:10.3109/14397595.2013.857582. PMID 24289197.

Template:WH Template:WS

Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

There are no established risk factors for eosinophilic granulomatosis with polyangiitis. However, certain environmental agents including various allergens, infections, desensitization, vaccination, and genetics may act as triggering agents. Those triggers may be responsible for the inflammatory response with eosinophils and lymphocytes and macrophages.

Risk Factors

Common Risk Factors

Common risk factors in the development of eosinophilic granulomatosis with polyangiitis may be various allergens, infections, genetic factors, and desensitization to drugs and vaccinations.

References

  1. ↑ Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, Garini G, Manganelli P, Bottero P, Tumiati B, Sinico RA, Savi M, Buzio C, Neri TM (September 2007). “HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome”. Arthritis Rheum. 56 (9): 3159–66. doi:10.1002/art.22834. PMID 17763415.
  2. ↑ Wieczorek S, Hellmich B, Arning L, Moosig F, Lamprecht P, Gross WL, Epplen JT (June 2008). “Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener’s granulomatosis”. Arthritis Rheum. 58 (6): 1839–48. doi:10.1002/art.23496. PMID 18512809.
  3. ↑ Gómez-Puerta JA, Gedmintas L, Costenbader KH (October 2013). “The association between silica exposure and development of ANCA-associated vasculitis: systematic review and meta-analysis”. Autoimmun Rev. 12 (12): 1129–35. doi:10.1016/j.autrev.2013.06.016. PMC 4086751. PMID 23820041.
  4. ↑ Puéchal X, Rivereau P, Vinchon F (July 2008). “Churg-Strauss syndrome associated with omalizumab”. Eur. J. Intern. Med. 19 (5): 364–6. doi:10.1016/j.ejim.2007.09.001. PMID 18549941.
  5. ↑ Orriols R, Muñoz X, Ferrer J, Huget P, Morell F (January 1996). “Cocaine-induced Churg-Strauss vasculitis”. Eur. Respir. J. 9 (1): 175–7. PMID 8834352.

Template:WH Template:WS

Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

Overview

Eosinophilic granulomatosis with polyangiitis develops through three phases, include prodromal phase, eosinophilic phase, vasculitic phase. Most complications result from the vasculitic phase. Most common complications include cardiomyopathy, myocardial infarction, perimyocarditis, rapidly progressive renal failure, GI bleeding, neuropathy, and status asthmaticus. Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated. Prognosis is most likely dependent on stage at which the disease was diagnosed and organ involvement. The five-factor score assessment (FFS) is a good predictor of survival rate. It can be used to choose the appropriate treatment.

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Five factor score 5 year mortality rate
0 11.9%
1 25.9%
>2 45.95%

References

  1. ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). “Churg-Strauss syndrome”. Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
  2. ↑ Aakerøy L, Amundsen BH, Skomsvoll JF, Haugen BO, Soma J (March 2011). “A 50-year-old man with eosinophilia and cardiomyopathy: need for endomyocardial biopsy?”. Eur J Echocardiogr. 12 (3): 257–9. doi:10.1093/ejechocard/jeq167. PMID 21138993.
  3. ↑ Lai RS, Lin SL, Lai NS, Lee PC (1998). “Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage”. Scand. J. Rheumatol. 27 (3): 230–2. PMID 9645420.
  4. ↑ Clutterbuck EJ, Evans DJ, Pusey CD (1990). “Renal involvement in Churg-Strauss syndrome”. Nephrol. Dial. Transplant. 5 (3): 161–7. PMID 2113641.
  5. ↑ Kaneki T, Kawashima A, Hayano T, Honda T, Kubo K, Koizumi T, Sekiguchi M, Ichikawa H, Matsuzawa K, Katsuyama T (February 1998). “Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer”. J. Gastroenterol. 33 (1): 112–6. PMID 9497232.
  6. ↑ Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ (April 2010). “Neurologic complications of Churg-Strauss syndrome–a prospective monocentric study”. Eur. J. Neurol. 17 (4): 582–8. doi:10.1111/j.1468-1331.2009.02902.x. PMID 20050889.
  7. ↑ Lestre S, Serrão V, João A, Pinheiro S, Lobo L (2009). “[Churg-Strauss syndrome presenting with cutaneous vasculitis]”. Acta Reumatol Port (in Portuguese). 34 (2A): 281–7. PMID 19569284.
  8. ↑ Guillevin L (October 2008). “Advances in the treatments of systemic vasculitides”. Clin Rev Allergy Immunol. 35 (1–2): 72–8. doi:10.1007/s12016-007-8068-4. PMID 18181034.
  9. ↑ Kim MY, Sohn KH, Song WJ, Park HW, Cho SH, Min KU, Kang HR (January 2014). “Clinical features and prognostic factors of Churg-Strauss syndrome”. Korean J. Intern. Med. 29 (1): 85–95. doi:10.3904/kjim.2014.29.1.85. PMC 3932399. PMID 24574837.
  10. ↑ 10.0 10.1 Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P (January 1996). “Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients”. Medicine (Baltimore). 75 (1): 17–28. PMID 8569467.
  11. ↑ Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). “The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort”. Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.
  12. ↑ Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL (June 2013). “A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients”. Ann. Rheum. Dis. 72 (6): 1011–7. doi:10.1136/annrheumdis-2012-201531. PMID 22887848.

Template:WH Template:WS

Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case#1


Suggested Reading

Suggested Reading

  1. Masi A, Hunder G, Lie J, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis angiitis). Arthritis Rheum. 1990;33:1094-1100
  2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187-92
  3. Leatherman J, Davies S, Hoidal J. Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders. Medicine. 1984;63:65-81
  4. Kallenberg CG. Antineutrophil cytoplasmic antibodies (ANCA) and vasculitis. Clin Rheumatol. 1990;9:S132-S35
  5. Weller PF, Plaut M, Taggart V, Trontell A. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report. J Allergy Clin Immunol. 2001;108:175-83
  6. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78:26-37
  7. Sehgal M, Swanson J, DeRemee R, Colby T. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc. 1995;70:337-41
  8. Lie J. Limited forms of Churg-Strauss syndrome. Pathol Annu. 1993;28:199-220
  9. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995;25:28-34
  10. Lane SE, Watts RA, Bentham G, Innes NJ, Scott DGI. Are environmental factors important in the aetiology of primary systemic vasculitis (abstract) ? Arthritis Rheum. 2001;44(suppl9):S57
  11. Watts RA, Lane SE, Scott DGI, Koldingsnes W, Nossent H, Gonzalez-Gay MA, Garcia-Porrua C, Bentham GA. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60:1156-7
  12. Fauci A, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978;89:660-76
  13. Frohnert P, Sheps S. Long term follow-up study of polyarteritis nodosa. Am J Med. 1967;43:8-14
  14. Guillevin L, Lê THD, Godeau P, Jaïs P, Wechsler B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol. 1988;27:258-64
  15. Guillevin L, Lhote F, Jarrousse B, Fain O. Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years. Ann Méd Interne (Paris). 1992;143:405-16
  16. Leib E, Restivo C, Paulus H. Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med. 1979;67:941-47
  17. Rose G, Spencer H. Polyarteritis nodosa. Q J Med. 1957;26:43-81
  18. Guillevin L, Fain O, Lhote F, et al. Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients. Arthritis Rheum. 1992;35:208-15
  19. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17-28
  20. Mahr A, Ayme S, Guillevin L. Prevalence and incidence of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis and Churg-Strauss syndrome in a french suburban population in 2000: a capture-recapture analysis (abstract). Arthritis Rheum. 2001;44(suppl9):S332
  21. Guillevin L, Guittard T, Bletry O, Godeau P, Rosenthal P. Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases. Lung. 1987;165:165-72
  22. Chumbley L, Harrison E, DeRemee R. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Mayo Clin Proc. 1977;52:477-84
  23. Haas C, Géneau C, Odinot J, et al. L’angéïte allergique avec granulome: syndrome de Churg et Strauss. Etude rétrospective de 16 observations. Ann Méd Interne (Paris). 1991;142:135-42
  24. Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D, Keystone E. Outcome of polyarteritis nodosa and Churg-Strauss syndrome. Arthritis Rheum. 1994;37:1798-1803
  25. Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M. Churg-Strauss syndome: outcome and long-term follow-up of 32 patients. Rheumatology. 2001;40:763-71
  26. Lai R, Lin S, Lee P. Churg-Strauss syndrome presenting with capillaritis and diffuse alveolar hemorrhage. Scand J Rheumatol. 1998;27:230-2
  27. Lhote F, Cohen P, Chemlal K, Jarrousse B, Guillevin L. Les manifestations pleurales au cours de la périartérite noueuse, de la maladie de Wegener et du lupus érythémateux systémique. Ann Méd Interne (Paris). 1992;143:228-32
  28. Herreman G, Ferme I, Puech H, Caubarrère I. Angéite granulomateuse de Churg et Strauss avec paralysie phrénique. Deux observations. Presse Méd. 1980;9:3631
  29. Cohen-Tervaert J, Kallenberg C. Neurologic manifestations of systemic vasculitides. Rheum Dis Clin North Am. 1993;19:913-40
  30. Ng K, Yeung H, Loo K, Chan H, Wong C, Li P. Acute fulminant neuropathy in a patient with Churg Strauss syndrome. Postgrad Med J. 1997;73:236-8
  31. Chang Y, Kargas S, Goates J, Horouplan D. Intraventricular and sub-arachnoid hemorrhage resulting from necrotizing vasculitis of the choroid plexus in a patient with Churg Strauss syndrome. Clin Neuropathol. 1993;12:84-7
  32. Davis M, Daoud M, McEvoy M, Su D. Cutaneous manifestations of Churg Strauss syndrome: a clinicopathologic correlation. J Acad Dermatol. 1997;37:199-203
  33. Finan M, Winkelmann R. The cutaneous extravascular necrotizing granuloma (Churg Strauss granuloma) and systemic disease: a review of 27 cases. Medicine (Baltimore). 1983;62:148-58
  34. Suen K, Burton J. The spectrum of eosinophilic infiltration of the gastrointestinal tract and its relationship to other disorders of angiitis and granulomatosis. Hum Pathol. 1979;10:31-43
  35. Guillevin L, Lhote F, Gallais V, et al. Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome. Ann Med Interne (Paris). 1995;146:260-7
  36. Sharma A, de Varennes B, Sniderman A. Churg-Strauss syndrome presenting with marked eosinophilia and pericardial effusion. Can J Cardiol. 1993;9:329-30
  37. Kozak M, Gill E, Green L. The Churg Strauss syndrome. A case report with angiographically documented coronary involvement and a review of the literature. Chest. 1995;107:578-80
  38. Isaka N, Araki S, Shibata M, al. e. Reversal of coronary artery occlusions in allergic granulomatosis and angiitis (Churg-Strauss syndrome). Am Heart J. 1994;128:609-13
  39. Hasley P, Follansbee N, Couleman J. Cardiac manifestations of Churg Strauss syndrome: report of a case and review of the literature. Am Heart J. 1990;120:996-9
  40. De Vlam K, De Keyser F, Goemaere S, Praet M, Veys E. Churg-Strauss syndrome presenting as polymyositis. Clin Exp Rheumatol. 1995;13:505-7
  41. Gaskin G, Clutterbuck E, Pusey C. Renal disease in the Churg Strauss syndrome. Diagnosis, management and outcome. Contrib Nephrol. 1991;94:58-65
  42. Guillevin L, Lhote F, Brauner M, Casassus P. Antineutrophil cytoplasmic antibodies (ANCA) and abnormal angiograms in polyarteritis nodosa and Churg-Strauss syndrome: indications for the diagnosis of microscopic polyangiitis. Ann Méd Interne (Paris). 1995;146:548-50
  43. Azar N, Guillevin L, Huong DL, Herreman G, Meyrier A, Godeau P. Symptomatic urogenital manifestations of polyarteritis nodosa and Churg-Strauss angiitis: analysis of 8 of 165 patients. J Urol. 1989;142:136-8
  44. Nissim F, Von der Valde J, Czernobilsky B. A limited form of Churg-Strauss syndrome. Ocular and cutaneous manifestations. Arch Pathol Lab Med. 1982;106:305-7
  45. Takanashi T, Uchida S, Arita M, Okada M, Kashii S. Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature. Ophtalmology. 2001;108:1129-33
  46. Généreau T, Lortholary O, Pottier MA, Michon-Pasturel U, Ponge T, de Wazieres B, Liozon E, Pinede L, Hachulla E, Roblot P, Barrier JH, Herson S, Guillevin L. Temporal artery biopsy : a diagnostic tool for systemic necrotizing vasculitis. Arthritis Rheum. 1999;42:2674-81
  47. Churg A, Brallas M, Cronin S, Churg J. Formes frustes of Churg Strauss syndrome. Chest. 1995;108:320-3
  48. Phanupak P, Kohler P. Onset of polyarteritis nodosa during allergic hyposensitization treatment. Am J Med. 1980;68:1866-72
  49. Drazen J, Israel E, O’Byrne P. Treatment of asthma with drugs modifying the leukotriene pathway. N Engl J Med. 1999;340:197-206
  50. Katz R, Papernik M. Zafirlukast and Churg-Strauss syndrome. JAMA. 1998;279:1949
  51. Knoell D, Lucas J, Allen J. Churg Strauss syndrome associated with zafirlukast. Chest. 1998;114:332-4
  52. Wechsler M, Garpestad E, Flier S, et al. Pulmonary infiltrates, eosinophilia and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998;279:455-7
  53. Guillevin L, Visser H, Noël LH, et al. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome: 62 patients. J Rheumatol. 1993;20:1345-9
  54. Cohen-Tervaert J, Goldschmeding R, Elema JD, von den Borne A, Kallenberg CG. Antimyeloperoxidase antibodies in the Churg Strauss syndrome. Thorax. 1991;46:70-1
  55. Cogan E, Schandené L, Crusiaux A, Cochaux P, Velu T, Goldman M. Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. N Engl J Med. 1994;330:535-8
  56. Stillwell T, Benson R. Cyclophosphamide-induced hemorrhagic cystitis: a review of 100 patients. Cancer. 1988;61:451-7
  57. Baker G, Kahl L, Zee B, Stolzer B, Agarwal A, Medsger TJ. Malignancy following treatment of rheumatoid arthritis with cyclophosphamide. Long-term case-control follow-up study. Am J Med. 1987;83:1-9
  58. Jarrousse B, Guillevin L, Bindi P, et al. Increased risk of Pneumocystis carinii pneumonia in patients with Wegener’s granulomatosis. Clin Exp Rheumatol. 1993;11:615-21
  59. Guillevin L, Lhote F, Cohen P, et al. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995;38:1638-45
  60. Gayraud M, Guillevin L, Cohen P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol. 1997;36:1290-7
  61. Luqmani R, Exley A, Kitas G, Bacon P. Disease assessment and management of the vasculitides. Baillere’s Clin Rheumatol. 1997;11:423-46
  62. Hamilos D, Christensen J. Treatment of Churg Strauss syndrome with high-dose intravenous immunoglobulins. J Allergy Clin Immunol. 1991;88:823-4
  63. Jayne DR, Lockwood CM. Intravenous immunoglobulins as sole therapy for systemic vasculitis. Br J Rheumatol. 1996;35:1150-3
  64. Henderson R, Hasleton P, Hamid B. Recurrence of Churg-Strauss vasculitis in a transplanted heart. Br Heart J. 1993;70:553
  65. McDermott E, Powell R. Cyclosporin in the treatment of Churg-Strauss syndrome. Ann Rheum Dis. 1998;57:256-7
  66. Tatsis E, Schnabel A, Gross W. Interferon alpha treatment in four patients with the Churg Strauss syndrome. Ann Intern Med. 1998;129:370-4
  67. Koutantji M, Pearce S, Harrold E. Psychological aspects of vasculitis. Rheumatol. 2000;39:1173-9
  68. Guillevin L, Cohen P, Larroche C, Queyrel V, Loustaud-Ratti V, Imbert B, Hausfater P, Ramanoelina J, Lacombe F, Roudier J, Bielefeld P, Petitjean P, Smadja D. The French Vasculitis Study Group. Treatment of HBV-related polyarteritis nodosa with lamivudine and plasma exchanges: a prospective, multicenter, pilot trial in 10 patients (abstract). Arthritis Rheum. 2001;44(suppl9):S271
External links

Template:Diseases of the musculoskeletal system and connective tissue


de:Churg-Strauss-Syndrom nl:Syndroom van Churg-Strauss


Template:WikiDoc Sources

Looking for the patient version?

Back to the patient-friendly article

Imprint

Privacy Policy

Terms and Conditions

© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH