Classification of Vasculitic Disorders

Size of Predominant Affected Vessels

Disorders

Symptoms and Signs

Large

Behçet disease

Giant cell arteritis

Takayasu arteritis

Limb claudication

Unequal blood pressure measurements or unequal pulse strength/absent pulse in the limbs

Central nervous system and retinal ischemic symptoms (eg, strokes)

Medium

Medium-vessel cutaneous vasculitis

Polyarteritis nodosa

Kawasaki disease

Symptoms of tissue infarction in affected organs, such as

  • Muscles: Myalgias

  • Nerves: Numbness, paresthesias, and/or weakness. Multiple mononeuropathy (mononeuritis multiplex) or polyneuropathy

  • Gastrointestinal tract: Abdominal pain, weight loss, and/or diarrhea. Mesenteric ischemia

  • Kidneys: New-onset hypertension (with renal artery involvement)

  • Skin: Ulcers, nodules, and livedo reticularis

Symptoms and signs in Kawasaki disease: Fever, rash, lymphadenopathy, conjunctivitis, and coronary artery aneurysms

Small

Eosinophilic granulomatosis with polyangiitis

Cryoglobulinemic vasculitis

Granulomatosis with polyangiitis

Immunoglobulin A–associated vasculitis (formerly called Henoch-Schönlein purpura)

Microscopic polyangiitis

Small-vessel cutaneous vasculitis

Symptoms of tissue infarction in affected organs similar to those for medium-sized vessels, except skin lesions more likely to be purpuric

In kidneys: Glomerulonephritis (usually asymptomatic)