[Churg-Strauss syndrome]

Presse Med. 2007 May;36(5 Pt 2):875-89. doi: 10.1016/j.lpm.2007.02.018. Epub 2007 Apr 3.
[Article in French]

Abstract

Churg-Strauss syndrome is a systemic and pulmonary vasculitis, defined by its association with severe asthma and with hypereosinophilia of the blood and tissues. The systemic vasculitis is a small-vessel vasculitis frequently associated with purpura, mononeuritis multiplex, and, more rarely, with rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage. Its prevalence of 7 to 13 per million population makes it one of the rarest of the systemic vasculitides. Anti-MPO (antimyeloperoxidase) pANCA (ANCA with a perinuclear fluorescence pattern) is present in 35-40% of cases and appears to determine a subgroup of patients with a higher frequency of renal damage, alveolar hemorrhage, and central nervous system damage. Cardiac involvement is an important cause of morbidity and the leading cause of mortality in Churg-Strauss syndrome. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to validated prognostic criteria (Five-Factor Score). Complete remission occurs in almost 90% of cases, and the 10-year survival rate has reached 79.4%. Relapses are frequent (25% of cases) and even after recovery from vasculitis, most patients (90%) still have asthma requiring corticosteroid treatment.

Publication types

  • Comparative Study
  • English Abstract
  • Review

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / administration & dosage
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Age Factors
  • Aged, 80 and over
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Child
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / drug therapy
  • Churg-Strauss Syndrome* / epidemiology
  • Churg-Strauss Syndrome* / etiology
  • Churg-Strauss Syndrome* / mortality
  • Churg-Strauss Syndrome* / therapy
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Infliximab
  • Interferon-alpha / therapeutic use
  • Male
  • Middle Aged
  • Plasma Exchange
  • Prevalence
  • Prognosis
  • Rituximab
  • Sex Factors

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunoglobulins, Intravenous
  • Immunologic Factors
  • Immunosuppressive Agents
  • Interferon-alpha
  • Rituximab
  • Cyclophosphamide
  • Infliximab