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Churg-strauss syndrome.

Loïc Guillevin1, Christian Pagnoux, Luc Mouthon

  • 1Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris V, Paris, France. loic.guillevin@cch.ap-hop-paris.fr

Seminars in Respiratory and Critical Care Medicine
|August 10, 2005
PubMed
Summary

Churg-Strauss syndrome (CSS) is a small-vessel vasculitis often starting with asthma. While corticosteroids are effective, many patients require long-term treatment for persistent asthma, with a 10-year survival rate of 79%.

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Area of Science:

  • Rheumatology
  • Immunology
  • Pulmonology

Background:

  • Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare small-vessel vasculitis.
  • It is characterized by asthma, hypereosinophilia, and systemic involvement, often affecting the heart, nerves, skin, and gastrointestinal tract.
  • The exact etiology remains unclear, though T-helper type 2 lymphocytes, eosinophils, and antineutrophil cytoplasm antibodies (ANCA) are implicated.

Purpose of the Study:

  • To describe the clinical features, diagnosis, and treatment outcomes of Churg-Strauss syndrome.
  • To highlight the role of asthma as a central feature and the impact of cardiac involvement on mortality.
  • To evaluate treatment response, remission rates, and long-term prognosis in CSS patients.

Main Methods:

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  • Review of clinical data from patients diagnosed with Churg-Strauss syndrome.
  • Analysis of demographic, clinical manifestations, biological features (including ANCA), and treatment strategies.
  • Evaluation of patient outcomes, including remission rates, relapse frequency, and survival.

    • Main Results:

    • CSS typically affects individuals around 50 years old, with asthma preceding systemic symptoms in most cases.
    • Common manifestations include sinusitis, pulmonary infiltrates, peripheral neuropathy, and skin lesions. Cardiac involvement is a major cause of mortality.
    • Hypereosinophilia is a key biological marker, and ANCA (particularly anti-MPO) are present in a significant proportion of patients. Corticosteroids are the primary treatment, with cyclophosphamide used for severe cases.

    Conclusions:

    • CSS is a complex vasculitis with significant morbidity and mortality, primarily due to cardiac involvement.
    • Early diagnosis and appropriate treatment, mainly with corticosteroids, lead to high remission rates (over 80%).
    • Persistent asthma often necessitates long-term corticosteroid therapy, and relapses occur in approximately 25% of patients.