Churg-Strauss syndrome: a retrospective study of 11 cases from a single center in Japan

Int J Rheum Dis. 2010 Aug;13(3):e6-e10. doi: 10.1111/j.1756-185X.2010.01479.x.

Abstract

Objective: To investigate the clinical characteristics of patients with Churg-Strauss syndrome (CSS), including symptoms, blood chemistry and immunological findings.

Patients and methods: We retrospectively investigated the records of 11 patients (six female and five male) with CSS admitted to our hospital from September 2003 to October 2009.

Results: Eight patients had preceding symptoms including bronchial asthma and allergic rhinitis. Seven patients showed eosinophilia. Nine patients had mononeuritis multiplex. Positive findings of myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) were found in five patients. Neither clinical manifestations nor laboratory findings were correlated with positivity for MPO-ANCA. However, the MPO-ANCA-positive group showed a higher level of blood urea nitrogen and proteinuria than those negative for MPO-ANCA. Ten patients recovered after starting steroid or immunosuppressive therapy, although one patient died of unknown etiology.

Conclusion: Although general assessments based on various factors such as medical history, clinical manifestation and laboratory studies are indispensable in CSS, MPO-ANCA might be useful as a predictor of renal dysfunction in patients with CSS.

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Asthma / immunology
  • Blood Urea Nitrogen
  • Churg-Strauss Syndrome* / blood
  • Churg-Strauss Syndrome* / drug therapy
  • Churg-Strauss Syndrome* / immunology
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Japan
  • Male
  • Middle Aged
  • Peroxidase / immunology
  • Proteinuria / immunology
  • Retrospective Studies
  • Rhinitis, Allergic, Perennial / immunology
  • Rhinitis, Allergic, Seasonal / immunology
  • Steroids / therapeutic use
  • Treatment Outcome
  • Young Adult

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Steroids
  • Peroxidase