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
|
β
|
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, HemoptysisΒ
|
| Eosinophilic granulomatosis with polyangiitis
(Churg-Strauss)[2]
|
+/-
|
–
|
–
|
+
|
–
|
–
|
–
|
+
|
Anti-MPO antibody (P-ANCA) (40%), Eotaxin-3
|
Eosinophilia, Anemia
|
β
|
βCr or BUN, Proteinuria, Erythrocyte casts, βIgE 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,Β Asthma, Urticarial rash
|
| Hydralazine-associated ANCA-associated vasculitis[3]
|
–
|
–
|
–
|
+
|
–
|
–
|
–
|
+/-
|
Anti-MPO antibody (P-ANCA), Anti-histone antibodies
|
Anemia
|
–
|
βCr 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,Β Anemia
|
β
|
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 depositsΒ
|
Histological confirmation
|
Acrocyanosis, Retinal hemorrhage, Purpura
|
| Hepatitis C virus-associated cryoglobulinemic vasculitis[5]
|
+/-
|
+/-
|
+
|
+
|
–
|
–
|
+
|
+/-
|
HCV RNA, Cryoglobulins
|
Leukocytosis,Β Anemia
|
β
|
βSerum 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-SchΓΆnlein purpura)[6]
|
–
|
–
|
+
|
+
|
–
|
–
|
–
|
–
|
IgA
|
Normochromic anemia, LeukocytosisΒ
|
β
|
Stool OB, βC3, βC4
|
–
|
–
|
Increased bowel wall thickness, hematomas, peritoneal fluid, and intussusception
|
Dilated loops of bowel consistentΒ 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
|
β
|
ANA, βC1q, βC3, βC4
|
–
|
–
|
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
|
β
|
Hypocomplementemia
|
–
|
Vascular stenosis and obstruction in visceral angiography
|
–
|
–
|
Perivascular inflammatory infiltrate of neutrophils with leukocytoclasia (releasing nuclear debris)
|
Histological confirmation
|
Palpable purpura, PetechiaeΒ
|
| Pulmonary disease
|
Langerhans Cell Histiocytosis[9]
|
+/-
|
–
|
–
|
+
|
–
|
–
|
–
|
–
|
CD1a,Β CD207,BRAF-V600E
|
Anemia
|
β
|
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
|
β
|
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, ReticulocytosisΒ
|
β
|
βLactate dehydrogenase (LDH), HypercalcemiaΒ
|
Thalami, brainstem, or cerebellum abnormality
|
Cerebral microangiopathy or hypertension
|
Hypoechoic kidneyΒ
|
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, ProteinuriaΒ
|
| Chronic Lymphocytic Leukemia (CLL)[12]
|
+
|
+
|
+
|
+
|
+/-
|
–
|
–
|
+/-
|
CD5, CD19, CD20,Β IgVH
|
Absolute lymphocytosis, Smudge cells
|
β
|
Flow cytometry
|
Staging
|
–
|
–
|
–
|
Large atypical cells, cleaved cells, and prolymphocytesΒ
|
Chromosomal and genetic testing
|
Easy bruising
|
| Multiple Myeloma[13]
|
+
|
–
|
+
|
+
|
+
|
+
|
+/-
|
+/-
|
Ig light chain
|
Anemia, Thrombocytopenia, Leukopenia
|
β
|
Bone marrow aspiration and biopsy, βCr
|
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,Β BCL2,Β BCL6, andΒ TP53
|
Lymphocytosis, Anemia, Thrombocytopenia
|
β
|
βLactate dehydrogenase (LDH), HypercalcemiaΒ
|
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
|
LeukopeniaΒ
|
β
|
Polyclonal gammopathy, βCr, 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
|
β
|
βD-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
|
Β 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]
|
+
|
+
|
+
|
+
|
–
|
–
|
–
|
+/-
|
Β IL-2 and IFN-Ξ³
|
Mild anemia
|
β
|
βACE, β1, 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
|
LeukocytosisΒ with left shift, Thrombocytosis
|
β
|
βD-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
|
Β Leukopenia, Lymphopenia, Anemia, Thrombocytopenia
|
β
|
βCr or BUN,
βALT 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
|
β
|
βCr or BUN,
βALT 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 stenosisΒ in CXR
|
Chondrolysis, Chondritis, Perichondritis
|
Clinical findings coupled with imaging
|
Ear pain and redness, Polyarthritis
|
References
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- β 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. PMCΒ 3565908. PMIDΒ 23316942.
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