Core Dermatology Diagnoses / CategoriesInflammatory

Systemic vasculitis

Author: Deputy Editor: Faculty Reviewer:

Publish date: Posted on
Last updated: October 24, 2023

Keywords #

systemic vasculitis
vasculitis
antineutrophil cytoplasmic antibodies
ANCA
palpable purpura

Diagnosis #

Systemic vasculitis is an inclusive term applied to a group of disorders that cause inflammation in blood vessel walls leading to subsequent ischemia and damage to the organs supplied by these vessels [1]. Vasculitis may occur idiopathically or as a secondary response to an underlying disease (e.g., hepatitis B infection) [2].  Classification is based on the size of the vessel affected [3].  The inflammatory process may be confined to one organ but may also involve several organ systems. Vasculitis should be considered in patients presenting with palpable purpura, pulmonary infiltrates, unexplained ischemic events, and multisystem disease [4]. The presence of antineutrophil cytoplasmic antibodies (ANCA) in the blood is an important diagnostic marker; however, there are also ANCA-negative vasculitis syndromes [5].

Key Concepts #
  • Systemic vasculitis encompasses a group of disorders that cause inflammation in blood vessel walls and subsequent ischemia and damage to the organs supplied by these vessels
  • May be idiopathic or secondary disease
  • Classification is primarily based upon the predominant size of the vessels involved
Epidemiology #

The most common type of vasculitis is Giant cell arteritis, in which the annual incidence reaches 15 to 35/100,000 aged > 50 years. Takayasu’s arteritis has a relatively uniform global incidence of one to two/million. The ANCA-associated vasculitides have an overall incidence of 20/million with a peak age of onset at 65 to 74 years [6].

Clinical Features #
  • Systemic symptoms such as fever, fatigue, weight loss, and arthralgias
  • Findings of a sensory and/or motor neuropathy
  • Palpable purpura
  • Absent, diminished, or tender pulses, bruits, or blood pressure discrepancies
Differential Diagnoses #

    Vasculitis mimickers

  • Infectious causes (e.g., endocarditis, HBV, HCV, HIV)
  • Atherosclerosis
  • Thromboembolic disease
  • Congenital causes (e.g., aortic coarctation, middle aortic syndrome)
  • Hereditary disorders (e.g., Marfan syndrome, Ehlers-Danlos syndrome)
  • Fibromuscular dysplasia
  • Hypercoagulable states (e.g., APS, TTP)
  • Other multisystem inflammatory disorders (e.g., sarcoidosis)
  • Malignancy
  • IgG4-related disease

    • Types of Vasculitis

  • Giant cell arteritis
  • Takayasu arteritis
  • Kawasaki syndrome
  • Polyarteritis nodosa
  • Thromboangiitis obliterans (Buerger disease)
  • Granulomatosis with polyangiitis (Wegener)
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
  • Microscopic polyangiitis
  • Immunoglobulin A vasculitis (Henoch-Schönlein purpura)
  • Cryoglobulinemic vasculitis
  • Behcet disease
Diagnostic Workup #

The diagnosis of the individual vasculitides is generally based on patterns of organ injury, the size of the vessels affected, histopathological features, and characteristic findings on diagnostic imaging. Diagnostic evaluation should include a detailed history, including drug use, infectious disease exposure, and symptoms of manifestations. Careful physical examination can identify potential sites of involvement of vasculitis and determine the extent of vascular lesions. Laboratory testing such as tests for antinuclear antibodies (ANA), complement levels, ANCA, UA to rule out nephritis, CBC, CMP to look for systemic involvement and biopsy for H&E and DIF (to rule out IgA mediate vasculitis).

Treatment #

If the vasculitis is secondary to an underlying disease, treatment of the underlying disease should be initiated. Immunosuppressive treatment is administered to stop vascular inflammation. Specific (e.g., antiviral drugs) or symptomatic (e.g., NSAID) management may be necessary.

Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/
References #
  1. Elefante, Elena, et al. “One Year in Review 2018: Systemic Vasculitis.” Clinical and Experimental Rheumatology, vol. 36 Suppl 111, no. 2, Apr. 2018, pp. 12–32.
  2. Sneller, Michael C., and Anthony S. Fauci. “Pathogenesis of Vasculitis Syndromes.” Medical Clinics of North America, vol. 81, no. 1, Jan. 1997, pp. 221–42. ScienceDirect, doi:10.1016/S0025-7125(05)70512-5.
  3. Rosenbaum, Richard B. “Chapter 50 – Connective Tissue Diseases, Vasculitis, and the Nervous System.” Aminoff’s Neurology and General Medicine (Fifth Edition), edited by Michael J. Aminoff and S. Andrew Josephson, Academic Press, 2014, pp. 1003–29. ScienceDirect, doi:10.1016/B978-0-12-407710-2.00050-3.
  4. Sharma, Poonam, et al. “Systemic Vasculitis.” American Family Physician, vol. 83, no. 5, Mar. 2011, pp. 556–65.
  5. Merkel, P. A., et al. “Prevalence of Antineutrophil Cytoplasmic Antibodies in a Large Inception Cohort of Patients with Connective Tissue Disease.” Annals of Internal Medicine, vol. 126, no. 11, June 1997, pp. 866–73. PubMed, doi:10.7326/0003-4819-126-11-199706010-00003.
  6. Watts, Richard A., and David G. I. Scott. “Epidemiology of the Vasculitides.” Seminars in Respiratory and Critical Care Medicine, vol. 25, no. 5, Oct. 2004, pp. 455–64. PubMed, doi:10.1055/s-2004-836139.
Contents