Trusted medical expertise in seconds.

Access 1,000+ clinical and preclinical articles. Find answers fast with the high-powered search feature and clinical tools.

Try free for 5 days
Evidence-based content, created and peer-reviewed by physicians. Read the disclaimer.

Vasculitides

Last updated: December 29, 2020

Summarytoggle arrow icon

Vasculitides are a heterogeneous group of autoimmune diseases, all characterized by inflammation of blood vessels (vasculitis) and subsequent ischemia and damage to the organs supplied by these vessels. Vasculitis may occur as a primary disease (idiopathic) or as a secondary response to an underlying disease (e.g., hepatitis B infection). Based on the size of the vessel affected, it can be classified into small-vessel, medium-vessel, or large-vessel vasculitis. While the inflammatory process may be confined to one organ, it may also involve several organ systems. Vasculitis should be considered in patients presenting with palpable purpura, pulmonary infiltrates, unexplained ischemic events, and multisystem disease. The detection of antineutrophil cytoplasmic antibodies (ANCA) in the blood is an important diagnostic marker; however, there are also ANCA-negative vasculitis syndromes. Immunosuppressive treatment is administered to stop vascular inflammation. Specific (e.g., antiviral drugs) or symptomatic (e.g., NSAIDs) management may be necessary. If the vasculitis is secondary to an underlying disease, treatment of the underlying disease should be initiated.

Giant cell arteritis (temporal arteritis)

See “Giant cell arteritis” for more information.

Takayasu arteritis (aortic arch syndrome)

In Takayasu arteritis, I can't TAKA YA pulse (pulseless disease).

Kawasaki disease (mucocutaneous lymph node syndrome)

See “Kawasaki disease” for more information.

Polyarteritis nodosa (PAN)

PAN should be considered in young adults presenting with stroke or myocardial infarction. The diagnosis may be confirmed with a biopsy of involved tissue.

In PAN, the Pulmonary Artery is Not involved, PANmural inflammation of the arterial wall is present, and PAN is often associated with hePANitis B.

Thromboangiitis obliterans

See “Thromboangiitis obliterans” for more information.

Granulomatosis with polyangiitis

See “Granulomatosis with polyangiitis” for more information.

Granulomatosis with polyangiitis is the 'C' disease: Curvy nose (saddle nose deformity), Chronic sinusitis, Cough, Conjunctivitis and Corneal ulceration, Cardiac arrhythmias, nonCaseating granulomas on biopsy, cANCA, Corticosteroids and Cyclophosphamide as treatment.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Churg-Strauss syndrome: pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules

Microscopic polyangiitis

The presentation of microscopic polyangiitis is similar to that of granulomatosis with polyangiitis, but it does not affect vessels in the upper respiratory tract (no sinusitis or rhinitis), does not involve granuloma formation, and is associated with pANCA (not cANCA).

Microscopic Polyangiitis has MyeloPeroxidase antibodies (i.e., pANCA).

On histopathology, the following leukocytoclastic vasculitis syndromes appear as nuclear debris from neutrophil leukocytes that have infiltrated blood vessel walls. [1]

Henoch-Schonlein purpura (immunoglobulin A vasculitis)

See “Henoch-Schonlein purpura” for more information.

Cryoglobulinemic vasculitis

The triad of arthralgia, palpable purpura, and fatigue is seen in ∼ 30% of patients with mixed cryoglobulinemia.

Cryoglobulinemia is caused by Cold-precipitable immunoglobulins and associated with hepatitis C.

Cutaneous small vessel vasculitis (formerly hypersensitivity vasculitis) [19]

Definition

A systemic vasculitis that is characterized by the deposition of immune complexes in arteries and veins of all sizes

Epidemiology [21]

  • Most common from the Mediterranean region to eastern Asia, with the highest prevalence observed in Turkey and Japan
  • Peak incidence: 20–40 years

Etiology

  • Autoimmune and infectious triggers (e.g., precipitating HSV or parvovirus infection) have been suggested. [22]
  • Strong HLA-B51 association

Clinical features [23][24]

Diagnostics [26][27]

PATHERGY: Positive pathergy test, Aphthous mouth ulcers, Thrombosis (arterial and venous), Hemoptysis (pulmonary artery aneurysm), Eye lesions (uveitis, retinal vasculitis), Recurrent Genital ulcers, Young at presentation (3rd decade)

Diagnostic criteria (International Study Group criteria)

  • Recurrent oral ulceration at least three times within a 12-month period
    AND
  • ≥ 2 of the following

Differential diagnosis

Treatment [27][28]

Clinical presentation Diagnostic clues Treatment

Giant cell arteritis

Takayasu arteritis
Kawasaki syndrome
  • High-dose ASA and IVIG
Polyarteritis nodosa
Thromboangiitis obliterans (Buerger disease)
  • Smoking cessation
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
Microscopic polyangiitis
lmmunoglobulin A
vasculitis
(Henoch-Schönlein purpura)
Cryoglobulinemic vasculitis
Cutaneous small vessel vasculitis
  • Drug-induced, infections
  • Discontinue drug intake
Behcet disease

References:[11]

  1. Alnaimat FA, Diamond HS. Behcet Disease. Behcet Disease. New York, NY: WebMD. http://emedicine.medscape.com/article/329099-overview. Updated: August 26, 2016. Accessed: August 4, 2017.
  2. Habibagahi M PhD, Habibagahi Z Md, Saidmardani SM Md, Sadeghian F Md. No Definite Association between Human Parvovirus B19 Infection and Behçet Disease.. Iranian journal of medical sciences. 2015; 40 (6): p.493-500.
  3. Jagdish R Nair, Robert J Moots. Behcet's disease. Clin Med (Northfield Il). 2017; 17 (1): p.71-77. doi: 10.7861/clinmedicine.17-1-71 . | Open in Read by QxMD
  4. Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease.. Pathology research international. 2012; 2012 : p.690390. doi: 10.1155/2012/690390 . | Open in Read by QxMD
  5. Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014; 121 : p.1703-1723. doi: 10.1016/B978-0-7020-4088-7.00110-3 . | Open in Read by QxMD
  6. West SG. Rheumatology Secrets. Elsevier Mosby ; 2014
  7. Behcet syndrome. https://www.dynamed.com/topics/dmp~AN~T114300/Behcet-syndrome#Overview-and-Recommendations. Updated: June 15, 2017. Accessed: November 30, 2017.
  8. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008; 67 (12): p.1656-1662. doi: 10.1136/ard.2007.080432 . | Open in Read by QxMD
  9. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  10. Tomelleri A, Campochiaro C, Sartorelli S, et al. AB0621 GENDER DIFFERENCES IN CLINICAL PRESENTATION AND VASCULAR PATTERN IN PATIENTS WITH TAKAYASU ARTERITIS. Abstracts accepted for Publication. 2019 . doi: 10.1136/annrheumdis-2019-eular.2407 . | Open in Read by QxMD
  11. Takayasu arteritis. https://medlineplus.gov/ency/article/001250.htm. . Accessed: November 9, 2020.
  12. Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol. 2002; 55 (7): p.481-486. doi: 10.1136/jcp.55.7.481 . | Open in Read by QxMD
  13. Bryl M, Guziński M, Rabczyński M, et al. Trudności w obrazowaniu choroby Takayasu – opis przypadku i przegląd literatury. Polish Journal of Radiology. 2012; 77 (4): p.67-71. doi: 10.12659/pjr.883633 . | Open in Read by QxMD
  14. Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of takayasu arteritis. Arthritis & Rheumatism. 1990; 33 (8): p.1129-1134. doi: 10.1002/art.1780330811 . | Open in Read by QxMD
  15. Ellen C. Ebert, Klaus D. Hagspiel, Michael Nagar, Naomi Schlesinger. Gastrointestinal Involvement in Polyarteritis Nodosa. Clinical Gastroenterology and Hepatology. 2008 .
  16. Polyarteritis Nodosa. https://rarediseases.org/rare-diseases/polyarteritis-nodosa/. . Accessed: November 9, 2020.
  17. Stanton M, Tiwari V. Polyarteritis Nodosa. StatPearls. 2018 .
  18. Stanson AW, Friese JL, Johnson CM, et al. Polyarteritis Nodosa: Spectrum of Angiographic Findings. RadioGraphics. 2001; 21 (1): p.151-159. doi: 10.1148/radiographics.21.1.g01ja16151 . | Open in Read by QxMD
  19. King TE. Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-features-and-diagnosis-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss.Last updated: July 8, 2016. Accessed: February 8, 2017.
  20. Assmann G, Molinger M, Pfreundschuh M, Bohle R, Zimmer V. Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment. SpringerPlus. 2014; 3 (1). doi: 10.1186/2193-1801-3-404 . | Open in Read by QxMD
  21. Ullah S, Ali M, Kyaw P, Nayeemuddin F, Sreedhar R, Gao J. P-ANCA negative eosinophilic granulomatosis with polyangiitis. Respiratory Medicine Case Reports. 2019; 27 : p.100830. doi: 10.1016/j.rmcr.2019.100830 . | Open in Read by QxMD
  22. Rose NR, Mackay IR. The Autoimmune Diseases. Academic Press ; 2006
  23. Chung SA, Seo P. Microscopic Polyangiitis. Rheumatic Disease Clinics of North America. 2010; 36 (3): p.545-558. doi: 10.1016/j.rdc.2010.04.003 . | Open in Read by QxMD
  24. Braun GS, Horster S, Wagner KS, Ihrler S, Schmid H. Cryoglobulinaemic vasculitis: classification and clinical and therapeutic aspects. Postgrad Med J. 2007; 83 (976): p.87-94. doi: 10.1136/pgmj.2006.046078 . | Open in Read by QxMD
  25. Mixed Cryoglobulinemia. https://rarediseases.org/rare-diseases/mixed-cryoglobulinemia/. . Accessed: November 8, 2020.
  26. Muchtar E, Magen H, Gertz MA. How I treat cryoglobulinemia. Blood. 2017; 129 (3): p.289-298. doi: 10.1182/blood-2016-09-719773 . | Open in Read by QxMD
  27. Baigrie D, Bansal P, Goyal A, Crane JS. Leukocytoclastic Vasculitis. StatPearls. 2020 .
  28. Manuel A, Victório T, Gomes C, Martins T, Neto AD. Vasculitis: an unusual manifestation in an HIV-infected patient. The Brazilian Journal of Infectious Diseases. 2015; 19 (4): p.439-441. doi: 10.1016/j.bjid.2015.04.006 . | Open in Read by QxMD
  29. Herold G. Internal Medicine. Herold G ; 2014