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Sarcoidosis and systemic vasculitis.

S R Fernandes1, B H Singsen, G S Hoffman

  • 1Department of Rheumatic and Immunologic Diseases and the Center for Vasculitis Care and Research, Cleveland Clinic Foundation, OH, USA.

Seminars in Arthritis and Rheumatism
|August 31, 2000
PubMed
Summary
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Systemic vasculitis is an uncommon complication of sarcoidosis, affecting various blood vessels. While corticosteroids offer palliative care, relapses and treatment-related morbidity are common in this condition.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Pathology

Background:

  • Sarcoidosis is a multisystem inflammatory disease characterized by non-caseating granulomas.
  • Systemic vasculitis is an unusual but recognized complication of sarcoidosis.
  • This study investigates six cases of sarcoidosis with concurrent systemic vasculitis.

Observation:

  • The six patients presented with systemic symptoms including fever, adenopathy, rash, pulmonary disease, musculoskeletal complaints, and ocular inflammation.
  • Biopsies confirmed sarcoidosis or necrotizing sarcoid granulomas in various tissues.
  • Arteriography revealed large vessel vasculitis in three African American patients, while white patients predominantly had small vessel vasculitis.

Findings:

  • Prior literature review included 14 adults and 8 children with sarcoid vasculitis, noting varied vessel involvement and racial predilections.

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  • African American and Asian patients were disproportionately represented in large vessel sarcoid vasculitis cases.
  • Four of the six patients responded to prednisone, but relapses occurred upon dose reduction.

    • Implications:

    • Sarcoid vasculitis can mimic other vasculitic syndromes, including hypersensitivity vasculitis and polyarteritis nodosa.
    • The condition affects small- to large-caliber vessels and shows racial disparities in large vessel involvement.
    • Corticosteroid and cytotoxic therapies are palliative, but relapses and treatment-related morbidity are frequent challenges.