Gastrointestinal Involvement in Dermatomyositis.

Area of Science:

• Rheumatology
• Gastroenterology
• Systemic Autoimmune Diseases

Background:

• Dermatomyositis is a systemic autoimmune disease primarily affecting skin, muscles, and lungs.
• Gastrointestinal (GI) involvement in dermatomyositis, though less common, can be severe and life-threatening.

Purpose of the Study:

• To characterize the clinical features of patients with severe GI involvement due to dermatomyositis.
• To review and synthesize data from a single center and existing medical literature.

Main Methods:

• Retrospective analysis of patients with GI compromise (erosions, ulcers, perforation, bleeding) from a single center.
• Comprehensive literature review of reported dermatomyositis GI cases from April 1990 to April 2021 via PubMed and Cochrane.

Main Results:

• Three patients from the center cohort experienced severe GI compromise, all women, presenting with abdominal pain and bleeding; all died.
• Literature review identified 50 cases; 77% were women, with abdominal pain as the main symptom. Mortality was 41.7%.
• Pathological findings in the center cohort revealed vascular ectasia, suggesting vasculopathy rather than vasculitis as the primary mechanism.

Conclusions:

• Severe gastrointestinal involvement in dermatomyositis indicates a poor prognosis and necessitates prompt, aggressive treatment.
• The underlying pathology appears to be a systemic vasculopathy affecting the GI tract, not a direct vasculitis.