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Severe gastrointestinal inflammation in adult dermatomyositis: characterization of a novel clinical association
- Source :
- The American journal of the medical sciences. 332(6)
- Publication Year :
- 2006
-
Abstract
- Object Gastrointestinal involvement in adult dermatomyositis (DM) and polymyositis (PM) is usually mild, resulting from myoenteric dismotility. Severe inflammation of the alimentary tract in cases of adult DM and PM is rare. The purpose of this study was to examine the prevalence and clinical characteristics of inflammatory gastrointestinal involvement in patients with DM. Methods The charts of all cases with polymyositis or dermatomyositis, registered in our rheumatology clinic between 1984 and 2004, were reviewed retrospectively for documentation of severe gastrointestinal involvement. The clinical course and the histopathologic findings in all the patients were noted, and the prevalence of this disorder was computed. Results Among 48 patients with DM or PM, 3 patients with DM and severe gastrointestinal tract manifestations were identified (6% of the study population). Edematous hyperemic bowel wall, with multiple erosions and ulcerous lesions were the prominent endoscopic findings, whereas diffuse mucosal inflammation and multiple vascular ectasias without vasculitis dominated the histologic picture. The resulting clinical course was notable for recurrent abdominal pain and bloody diarrhea, ending catastrophically in two patients with fatal gastrointestinal perforations, despite aggressive immunosuppressive therapy. Conclusions Severe inflammatory gastrointestinal tract disease should be recognized as a grave, albeit rare, manifestation of adult DM that portends a poor prognosis and carries a high rate of fatal complications. The role of vasculopathy in the pathogenesis of this syndrome remains to be determined.
- Subjects :
- Adult
medicine.medical_specialty
Pathology
Gastrointestinal Diseases
Prednisolone
Perforation (oil well)
Anti-Inflammatory Agents
Gastroenterology
Polymyositis
Dermatomyositis
Fatal Outcome
Internal medicine
Azathioprine
medicine
Humans
Cyclophosphamide
Inflammation
Gastrointestinal tract
Vascular disease
business.industry
Immunoglobulins, Intravenous
General Medicine
medicine.disease
Connective tissue disease
Adult dermatomyositis
Methotrexate
Female
business
Vasculitis
Immunosuppressive Agents
Hydroxychloroquine
Subjects
Details
- ISSN :
- 00029629
- Volume :
- 332
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- The American journal of the medical sciences
- Accession number :
- edsair.doi.dedup.....cfa0577d26c241269127e04c10b96fbc