Vasculitides are a heterogeneous group of rare autoimmune diseases characterized by blood vessel inflammation (vasculitis). Inflammation can lead to ischemia, necrosis, and/or hemorrhage, with subsequent end-organ damage. Vasculitides are either primary (idiopathic) or secondary to an underlying disease (e.g., HBV infection, cancer, systemic lupus erythematosus) or drug use. Vasculitides are further classified based on the size of the affected vessels: small-, medium-, or large-vessel vasculitis, or variable vessel vasculitis. Diagnosing vasculitides is often challenging, as symptoms are usually nonspecific; vasculitides should be considered in patients presenting with constitutional symptoms and signs of multisystem disease (e.g., palpable purpura, pulmonary infiltrates, unexplained ischemic events). Laboratory studies, imaging, and histopathology are often required to confirm the diagnosis. Management should involve a multidisciplinary team (e.g., rheumatology, ophthalmology, neurology) and aims to promptly prevent the progression of vascular inflammation with immunosuppressive therapy to avoid organ damage and death. Treatment of the underlying cause (e.g., with antiviral drugs) and/or symptomatic management (e.g., with NSAIDs) is often necessary.
- Primary (idiopathic)
Secondary to another disease or drug use, e.g.:
- Infectious diseases
- Drugs: e.g., hydralazine, cocaine
- Malignancy: e.g., multiple myeloma, lymphoproliferative disorders
- Autoimmune diseases: e.g., systemic lupus erythematosus (SLE), Sjogren syndrome, sarcoidosis, IBD
- Based on the 2012 Chapel Hill Consensus Nomenclature
- Classification is based on the size of the vessel predominantly affected:
Approach to management 
Vasculitis should be suspected in patients with constitutional symptoms and multisystemic inflammatory disease. Specific manifestations depend on the vessels affected.
- Tailor the approach to the suspected disease and/or affected organ or system.
- Rule out other (more common) diagnoses (e.g., infections, cancer, other autoimmune diseases).
- Assess for secondary causes of vasculitis (see “Etiology”).
- Consult a rheumatologist and/or other specialists as required.
- Start management based on disease severity and the affected organ or system.
- Perform a thorough history and physical examination; ≥ 1 of the following clinical features are usually found in patients with vasculitis :
- Findings suggestive of vasculitis on routine studies include:
- CBC: anemia of chronic disease, thrombocytosis, eosinophilia
- Inflammatory markers: ↑ ESR, ↑ CRP
- Infectious diseases workup: positive hepatitis B testing or hepatitis C testing
- Autoimmune diseases workup: negative ANAs and aPL antibodies
- Chest x-ray: lung involvement (e.g., ground glass opacity, nodular lesions)
Vasculitis has a wide variety of signs and symptoms; there is no single typical presentation.
Consider further diagnostics guided by clinical suspicion to:
- Determine the extent of the disease, e.g.:
- Establish the type of vasculitis: e.g., ↓ ANCAs and ↑ cryoglobulins indicates cryoglobulinemic vasculitis
- Confirm the diagnosis: with imaging (e.g., MRA, CTA) or histological studies
|Overview of large-vessel vasculitides|
|Overview of medium-vessel vasculitides|
ANCA-associated small-vessel vasculitis
|Overview of ANCA-associated small-vessel vasculitides|
Granulomatosis with polyangiitis is the 'C' disease: Curvy nose (saddle nose deformity), Chronic sinusitis, Cough, Conjunctivitis and Corneal ulceration, Cardiac arrhythmias, non-Caseating granulomas on biopsy, cANCA, Corticosteroids and Cyclophosphamide as treatment.
Non-ANCA-associated small-vessel vasculitis
|Overview of non-ANCA-associated small-vessel vasculitides|
|Cutaneous small-vessel vasculitis |
Variable vessel vasculitis
|Overview of variable vessel vasculitides|
Cogan syndrome