Churg-Strauss syndrome: Update on clinical, laboratory and therapeutic aspects

Karina A. Keogh, Ulrich Specks

Research output: Contribution to journalReview articlepeer-review

20 Scopus citations

Abstract

Originally described over fifty years ago as a disorder of asthma, eosinophilic inflammation and small vessel vasculitis, Churg-Strauss syndrome is now defined as one of the ANCA-associated vasculitides. The predilection of disease manifestations for the respiratory tract, preferred affliction of small vessels including capillaries, and the frequent occurrence of anti-neutrophil cytoplasmic antibodies (ANCA) justify this grouping together with Wegener's granulomatosis and microscopic polyangiitis. However, the allergic background in which the vasculitis presents, typically characterized by asthma and prominent peripheral blood and tissue eosinophilia, render it unique among the primary systemic vasculitis syndromes. Despite recent interest in a potential link between leukotriene receptor antagonist use for asthma and the onset of Churg-Strauss syndrome, it remains a rare disease with poorly understood pathogenesis. This review provides an update on the clinical diagnosis of Churg-Strauss syndrome in light of changing disease definitions and classifications, and focuses on evolving therapeutic approaches for this challenging systemic disorder.

Original languageEnglish (US)
Pages (from-to)3-12
Number of pages10
JournalSarcoidosis Vasculitis and Diffuse Lung Diseases
Volume23
Issue number1
StatePublished - Mar 1 2006

Keywords

  • ANCA
  • Asthma
  • Churg-Strauss syndrome
  • Eosinophilia
  • Leukotriene receptor antagonists
  • Vasculitis

ASJC Scopus subject areas

  • Internal Medicine
  • Immunology and Allergy
  • Pulmonary and Respiratory Medicine

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