The Science Journal of the American Association for Respiratory Care

Case Reports

February 2002 / Volume 47 / Number 2 / Page 167

Churg-Strauss Syndrome Occurring 30 Years After the Onset of Ulcerative Colitis

Athanasios A Prekates MD PhD, Stylianos E Orfanos MD PhD, Christina J Routsi MD PhD, Aikaterini Ch Pantelidaki MD PhD, and Charis S Roussos MD PhD

Churg-Strauss syndrome (CSS) is a rare pulmonary and systemic vasculitis associated with asthma, with peripheral blood and/or tissue eosinophilia. We report the case of a 53-year-old woman who was admitted to the hospital with pneumonia and coma secondary to right hemisphere intracerebral hemorrhage. Although she recovered from the pneumonia, she remained comatose and had sinusitis and persisting blood eosinophilia. A muscle biopsy revealed eosinophilic vasculitis. The diagnosis of CSS was made and the patient recovered after being treated with prednisone and cyclophosphamide. This case reports the very rare appearance of CSS 30 years after the first appearance of ulcerative colitis and 27 years after the onset of asthma.
Key words: Churg-Strauss syndrome, CSS, ulcerative colitis, asthma, systemic vasculitis.
[Respir Care 2002;47(2):167–170]

Introduction

Churg-Strauss syndrome (CSS) is a rare disease that represents 2.2% of all vasculitis cases. CSS is characterized by asthma, peripheral blood and/or tissue eosinophilia, and evidence of systemic vasculitis. CSS appears to emerge with a highly variable delay after the onset of asthma, and it has rarely been described in association with ulcerative colitis or other inflammatory bowel diseases. We report the case of a 53-year-old woman with longstanding ulcerative colitis, asthma, allergic rhinitis, sinusitis, peripheral eosinophilia (> 1,500 cells/mL), central nervous system involvement, and eosinophilic vasculitis on muscle biopsy, and who developed CSS 30 years after the first appearance of ulcerative colitis.

The entire text of this article is available in the printed version of the February 2002 RESPIRATORY CARE.

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