Gastrointestinal Involvement in Dermatomyositis

Abstract

Dermatomyositis is a systemic vasculopathy mainly affecting skin, muscle and lung, but may affect the gastrointestinal tract. We aim to describe clinical characteristics of patients with severe gastrointestinal involvement related to dermatomyositis in our center and medical literature. We retrospectively analysed these patients in our center, including cases of erosions/ulcers, perforation or digestive bleeding. Reported cases from April 1990 to April 2021 were reviewed through PubMed and Cochrane. From our cohort (n = 188), only 3 presented gastrointestinal compromise. All were women (10, 46 and 68 years). The initial symptom was abdominal pain and all had ≥2 episodes of digestive bleeding. All died due to complications of gastrointestinal involvement. Available pathological samples showed vascular ectasia. From the literature review (n = 50), 77% were women with a mean age of 49 years and the main symptom was abdominal pain (65%). All presented active muscular and cutaneous involvement at complication diagnosis. Mortality was 41.7%. The underlying lesion was perforation or ulcer (n = 22), intestinal wall thickening (n = 2), macroscopic inflammation (n = 2) or intestinal pneumatosis (n = 15). In 13 cases, vasculitis was described. Gastrointestinal involvement in dermatomyositis denotes severity, so an early intensive treatment is recommended. Pathological findings suggest that the underlying pathophysiological mechanism is a vasculopathy and not a true vasculitis.

Keywords: dermatomyositis; gastrointestinal bleeding; gastrointestinal involvement; gastrointestinal perforation; intestinal pneumatosis.

Figure 1

(A) (case 1): Plastic-embedded semithin cross-section muscle biopsy. Note the loss of capillaries in the peripheral area with mega-capillaries (star). (B) (case 2): Ulcerated mucosa area lined by granulation tissue, vascular ectasia and acute fibrinoleukocyitary inflammation. Some areas of the oesophageal wall with a moderate atrophy of the inner muscular layer with slight fibrosis and oedema. (C) (case 2) Mucosal inflammatory infiltrate with vascular ectasia extending to submucosa. Decreased glandular acini occupied by fibrin. (D) (case 3): Antral staking erythematosus without erosions or ulcerations evident. In the duodenum (from bulb to third duodenal portion), fibrin-coated erosions and well demarcated erythematous lesion with peripheral oedema is seen, without evidence of active bleeding.

Figure 2

Appearance of the heliotrope rash coinciding with the onset of digestive symptoms in case 1.

Figure 3

Clinical-epidemiological features of GI involvement in DM.