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

Severe Abdominal Manifestations in Juvenile Dermatomyositis.

Author information

1
Laboratoire d'Immunogénétique des maladies auto-immunes de l'enfant, Institut Imagine, INSERM U 1163.
2
Université de Paris.
3
Service de Neurophysiologie clinique, Centre de Référence des Maladies Neuromusculaires.
4
Service de Gastroentérologie et d'Hépatologie pédiatrique.
5
UF d'Hépatologie pédiatrique, Centre National de Référence Maladies Rares AVB-Cholestases génétiques, Hôpital Necker, APHP.
6
Equipe Contrôle de la croissance cellulaire par les nutriments, Institut Necker Enfants-Malades, INSERM U1151.
7
Service d'anatomo-pathologie.
8
Service d'Immunologie, Hématologie et Rhumatologie pédiatrique, centre de référence national des maladies rhumatologiques et systémiques auto-immunes rares pédiatriques (RAISE).
9
Service de Dermatologie et Dermatologie Pédiatrique.
10
Service d'Imagerie Pédiatrique, Hôpital Necker, APHP, Paris, France.

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.

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