MyEClinic
MyEClinic
Health Dictionary Find a Doctor

Small-sized vessel vasculitis with arthralgia


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

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
Small-Vessel Vasculitis ANCA-associated vasculitis Microscopic polyangiitis[1] + – – +/- – – – + 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 
Eosinophilic granulomatosis with polyangiitis

(Churg-Strauss)[2]

+/- – – + – – – + 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[3] – – – + – – – +/- 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 Cryoglobulinemic vasculitis[4] +/- +/- – + +/- – – – 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[5] +/- +/- + + – – + +/- 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)[6] – – + + – – – – 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)[7] – – +/- + – – – – C1q Mild anemia ↑ ANA, ↓C1q, ↓C3, ↓C4 – – Hepatomegaly, Splenomegaly – Deposits of immunoglobulins, complement, or fibrin around blood vessels Urticaria,

Histological confirmation

Urticaria, Hematuria
Cardiovascular disease Leukocytoclastic Vasculitis[8] + – – + – – – + 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[9] +/- – – + – – – – 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
Renal disease Acute Poststreptococcal Glomerulonephritis[10] – – – + – – + +/- 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[11] + + – + – – + + 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)[12] + + + + +/- – – +/- 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[13] + – + + + + +/- +/- 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
Non-Hodgkin Lymphoma[14] + + + + +/- +/- +/- +/- 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[15] + + – +/- +/- – – +/- 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[16] +/- + – +/- – – + + 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[17] + +/- – + – – – + 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[18] + + + + – – – +/-  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[19] + + + + – – – +/- 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[20] + + + + – – + + 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[21] – – + + + + – – 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[22] – +/- +/- + + – – – – 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

References

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ 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.
  4. ↑ Ferri C, Mascia MT (January 2006). “Cryoglobulinemic vasculitis”. Curr Opin Rheumatol. 18 (1): 54–63. PMID 16344620.
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 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.
  9. ↑ Margo CE, Goldman DR (2008). “Langerhans cell histiocytosis”. Surv Ophthalmol. 53 (4): 332–58. doi:10.1016/j.survophthal.2008.04.007. PMID 18572052.
  10. ↑ 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.
  11. ↑ Corrigan JJ, Boineau FG (November 2001). “Hemolytic-uremic syndrome”. Pediatr Rev. 22 (11): 365–9. PMID 11691946.
  12. ↑ 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.
  13. ↑ Michels TC, Petersen KE (March 2017). “Multiple Myeloma: Diagnosis and Treatment”. Am Fam Physician. 95 (6): 373–383. PMID 28318212.
  14. ↑ 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.
  15. ↑ Lin RY (January 1986). “Serum sickness syndrome”. Am Fam Physician. 33 (1): 157–62. PMID 2867672.
  16. ↑ Venugopal A (2014). “Disseminated intravascular coagulation”. Indian J Anaesth. 58 (5): 603–8. doi:10.4103/0019-5049.144666. PMC 4260307. PMID 25535423.
  17. ↑ 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.
  18. ↑ 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.
  19. ↑ Murdoch DR (January 2003). “Diagnosis of Legionella infection”. Clin. Infect. Dis. 36 (1): 64–9. doi:10.1086/345529. PMID 12491204.
  20. ↑ Tsokos, George C. (2011). “Systemic Lupus Erythematosus”. New England Journal of Medicine. 365 (22): 2110–2121. doi:10.1056/NEJMra1100359. ISSN 0028-4793.
  21. ↑ Scott JT (1991). “The gold standard in rheumatoid arthritis”. J R Soc Med. 84 (9): 513–4. PMC 1293405. PMID 1682491.
  22. ↑ Emmungil H, Aydın SZ (2015). “Relapsing polychondritis”. Eur J Rheumatol. 2 (4): 155–159. doi:10.5152/eurjrheum.2015.0036. PMC 5047229. PMID 27708954.

Template:WH Template:WS

Imprint

Privacy Policy

Terms and Conditions

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