Vasculitis diagnostic criteria

Vasculitis
Overview
Classification Large-sized vessel vasculitis Takayasu's Arteritis Temporal Arteritis Medium-sized vessel vasculitis Kawasaki's Disease Polyarteritis Nodosa Small-sized vessel vasculitis Churg-Strauss Syndrome Cutaneous leukocytoclastic vasculitis Essential cryoglobulinemic vasculitis Henoch-Schonlein Purpura Microscopic polyangiitis Wegener's Granulomatosis Variable-sized vessel vasculitis Sjogren syndrome Cogan syndrome Single organ vasculitis Primary central nervous system angiitis
Causes
Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: M.Umer Tariq [2]

Diagnostic Criteria

Takayasu arteritis

At least 3 out of 6 criteria yields sensitivity and specificity of 90.5 and 97.8%:

• Onset < 40 years
• Claudication of extremities
• Decreased pulsation of one or both brachial arteries
• At least 10 mmHg systolic difference in both arms
• Bruit over one or both carotid arteries or abdominal aorta
• Arteriographic narrowing of aorta, its primary branches, or large arteries in upper or lower extremities

Giant cell (temporal) arteritis

At least 3 out of 5 criteria yields sensitivity and specificity of 95 and 91%:

• Age at onset ≥ 50 years
• New onset headache with localized tenderness
• Temporal artery tenderness or decreased pulsation
• Elevated ESR ≥ 50 mm/hour Westergren
• Temporal artery biopsy showing vasculitis with mononuclear cell infiltrate or granulomatous inflammation, usually with multinucleated giant cells

Polyarteritis nodosa

At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%:

• Unexplained weight loss > 4 kg
• Livedo reticularis
• Testicular pain
• Myalgias, weakness
• Mononeuropathy or polyneuropathy
• New onset diastolic blood pressure > 90 mmHg
• Elevated serum BUN (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL)
• Hepatitis B infection
• Arteriographic abnormalities
• Arterial biopsy showing polymorphonuclear cells

Wegener's granulomatosis

At least 2 out of 4 criteria yields sensitivity and specificity of 88 and 92%.

• Nasal or oral inflammation (oral ulcers or purulent/bloody nasal discharge, may be painful)
• Abnormal CXR showing nodules, infiltrates, cavities
• Microscopic hematuria or RBC casts
• Vessel biopsy shows granulomatous inflammation

Kawasaki disease

Diagnosis requires fever lasting five days or more with at least 4 out of 5 criteria:

• Bilateral conjunctival injection
• Injected or fissured lips, injected pharynx, or strawberry tongue
• Erythema of palms/soles, edema of hands/feet, periungual desquamation
• Polymorphous rash
• Cervical lymphadenopathy (at least one node > 1.5 cm)

Isolated CNS vasculitis

Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy.

Churg-Strauss arteritis

At least 4 criteria yields sensitivity and specificity of 85 and 99.7%.

• Asthma (history of wheezing or presently wheezing)
• Eosinophilia > 10% on CBC
• Mononeuropathy or polyneuropathy
• Migratory or transient pulmonary opacities on CXR
• Paranasal sinus abnormalities
• Vessel biopsy showing eosinophils in extravascular areas

Hypersensitivity vasculitis

At least 3 out of 5 criteria yields sensitivity and specificity of 71 and 84%:

• Age > 16
• Use of possible triggering drug in relation to symptoms
• Palpable purpura
• Maculopapular rash
• Skin biopsy showing neutrophils around vessel

Henoch-Schonlein purpura

Presence of 3 or more criteria yielded sensitivity of 87% while less than 2 criteria yielded hypersensitivity vasculitis in 74%:

• Palpable purpura (usually of buttocks & legs)
• Mesenteric ischemia
• GI bleed
• Hematuria
• Onset < 20 years
• No new medications