Last updated: June 9, 2023

Summarytoggle arrow icon

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.

General principlestoggle arrow icon

Etiology [1]

Classification [2]

Approach to management [1]

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.

Most patients with vasculitis present with nonspecific features (e.g., constitutional symptoms, elevated inflammatory markers).

Initial evaluation

Vasculitis has a wide variety of signs and symptoms; there is no single typical presentation.

Additional evaluation

Consider further diagnostics guided by clinical suspicion to:


Large-vessel vasculitistoggle arrow icon

Overview of large-vessel vasculitides
Clinical features Diagnostics Management
Giant cell arteritis [3]
Takayasu arteritis [3][5]

Medium-vessel vasculitistoggle arrow icon

Overview of medium-vessel vasculitides
Clinical features Diagnostics Management
Kawasaki disease
Polyarteritis nodosa

Small-vessel vasculitistoggle arrow icon

ANCA-associated small-vessel vasculitis

Overview of ANCA-associated small-vessel vasculitides
Clinical features Diagnostics Management

Granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis

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
Clinical features Diagnostics Management
IgA vasculitis
Cryoglobulinemic vasculitis
Cutaneous small-vessel vasculitis [7][8]

Variable vessel vasculitistoggle arrow icon

Overview of variable vessel vasculitides
Clinical features Diagnostics Management
Behcet disease

Cogan syndrome [2][9]

Differential diagnosestoggle arrow icon

The differential diagnoses listed here are not exhaustive.

Referencestoggle arrow icon

  1. Younger DS. Overview of the Vasculitides. Neurol Clin. 2019; 37 (2): p.171-200.doi: 10.1016/j.ncl.2019.01.005 . | Open in Read by QxMD
  2. Espígol-Frigolé G, Prieto-González S, Alba MA, et al. Advances in the Diagnosis of Large Vessel Vasculitis. Rheumatic Disease Clinics of North America. 2015; 41 (1): p.125-140.doi: 10.1016/j.rdc.2014.10.001 . | Open in Read by QxMD
  3. Kim ESH, Beckman J. Takayasu arteritis: challenges in diagnosis and management.. Heart. 2018; 104 (7): p.558-565.doi: 10.1136/heartjnl-2016-310848 . | Open in Read by QxMD
  4. Bardi M, Diamantopoulos AP. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary. Radiol Med (Torino). 2019; 124 (10): p.965-972.doi: 10.1007/s11547-019-01058-0 . | Open in Read by QxMD
  5. Suresh E. Diagnostic approach to patients with suspected vasculitis. Postgrad Med J. 2006; 82 (970): p.483-488.doi: 10.1136/pgmj.2005.042648 . | Open in Read by QxMD
  6. Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis & Rheumatism. 2012; 65 (1): p.1-11.doi: 10.1002/art.37715 . | Open in Read by QxMD
  7. Micheletti RG, Pagnoux C. Management of cutaneous vasculitis. Presse Med. 2020; 49 (3): p.104033.doi: 10.1016/j.lpm.2020.104033 . | Open in Read by QxMD
  8. Goeser MR, Laniosz V, Wetter DA. A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-Vessel Vasculitis. Am J Clin Dermatol. 2014; 15 (4): p.299-306.doi: 10.1007/s40257-014-0076-6 . | Open in Read by QxMD
  9. A. Greco, A. Gallo, M. Fusconi, G. Magliulo, R. Turchetta, C. Marinelli, G.F. Macri, A. De Virgilio, M. de Vincentiis. Cogan's syndrome: An autoimmune inner ear disease. Autoimmun Rev. 2013; 12 (3): p.396-400.doi: 10.1016/j.autrev.2012.07.012 . | Open in Read by QxMD

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