Severe Abdominal Manifestations in Juvenile Dermatomyositis

J Pediatr Gastroenterol Nutr. 2020 Feb;70(2):247-251. doi: 10.1097/MPG.0000000000002575.

Abstract

Juvenile dermatomyositis (JDM) is a rare and heterogeneous pediatric-onset idiopathic inflammatory myopathy. Gastrointestinal (GI) involvement occurs in 22% to 37% of JDM patients but has only been described in case reports. In this retrospective, single-center, observational study, we aimed to assess the causes and management of severe GI manifestations in JDM patients. We studied a cohort of 9 patients among 110 JDM patients followed during the study period (8.3%). The GI complications were related to JDM in most cases (17/19), with digestive tract involvement (n = 10), acute pancreatitis (n = 4), and hepatitis (n = 3). Three patients died from refractory JDM 2.9 years (2-3.6) after the JDM diagnosis. We highlight the need to consider pancreatitis as a main diagnostic factor in JDM patients with severe GI manifestations and the requirement of early aggressive treatment for these patients.

Publication types

  • Observational Study

MeSH terms

  • Acute Disease
  • Child
  • Cohort Studies
  • Dermatomyositis* / complications
  • Dermatomyositis* / diagnosis
  • Humans
  • Pancreatitis* / diagnosis
  • Pancreatitis* / etiology
  • Retrospective Studies