[Gastrointestinal manifestations in idiopathic inflammatory myopathies : Dysphagia, intestinal vasculopathy and pneumatosis cystoides intestinalis].

In idiopathic inflammatory myopathies (IIM), gastrointestinal manifestations are rare and poorly recognized apart from the relatively common dysphagia. Oropharyngeal or esophageal dysphagia in IIM is associated with aspiration and malnutrition and responds well to conventional immunomodulation except for inclusion body myositis. The rare intestinal vasculopathy in nuclear matrix protein 2 (NXP2)-associated dermatomyositis affects both children and adults, causing life-threatening perforation and bleeding from the esophagus to the colon with a high mortality. It responds poorly to standard immunomodulation. Experimental treatment approaches with Janus kinase (JAK) inhibitors (by interferon inhibition), vedolizumab, and the C5 inhibitor eculizumab have recently shown promising results. Pneumatosis cystoides intestinalis, also a rarity, manifests as gas-filled submucosal cysts primarily in the small and large bowels and is often but not always an asymptomatic incidental finding in IIM amenable to conservative management. The IIM can involve dysmotility and hypomotility throughout the gastrointestinal tract, potentially leading to intestinal pseudo-obstruction and bacterial overgrowth with malnutrition. Notably, elevated aspartate aminotransferase (AST) and alanine transaminase (ALT) in IIM with high creatine kinase typically reflect skeletal muscle release rather than the manifestation of a hepatopathy.