P1302 - Severe Malnutrition Secondary to Juvenile Dermatomyositis: A Rare Case Report

Patrick Tempera, DO¹, Skyelor Black, BS², Nour Parsa, MD^2
1Albany Medical Center, Albany, NY; ²Loma Linda University Health, Loma Linda, CA

Introduction: Juvenile Dermatomyositis (JDM) is a rare, chronic inflammatory myopathy often thought to be an autoimmune disease process. It typically presents early in life with proximal muscle weakness. Gastrointestinal abnormalities, when present, manifest with symptoms such as dysphagia. Lower gastrointestinal problems involving the small and large intestine are quite rare and usually present as late manifestations. We describe a case of JDM with significant lower gastrointestinal involvement leading to severe malnutrition.

Case Description/Methods: A 25-year-old male with JDM complicated by diffuse subcutaneous calcinosis, presented with a three-year history of progressively worsening difficulty swallowing of solids and unintentional weight loss. On admission, the patient had abdominal pain, bloating, alternating constipation with diarrhea, loss of appetite, and unintentional weight loss over 2 months. The patient’s weight 3 years prior was 115 lbs but had decreased to 65 lbs. Swallowing studies showed uncoordinated swallowing and gross aspiration. Upper endoscopy could not be performed due to oral JDM involvement preventing placement of a bite block. He received gastrojejunal tube placement for venting and feeds. His clinical condition continued to decline, and he had residual output of up to 500 ml. Changes in feeding frequency and rate provided no improvement and his residual output increased to over 1000 mL per day despite initiating promotility agents. It was thought that the patient was experiencing severe small and large intestinal dysmotility due to JDM. Unfortunately, our patient suffered a large volume aspiration event, and he was transferred to the intensive care unit.

Discussion: JDM with lower gastrointestinal manifestations is a rare complication with significant morbidity and mortality. Early diagnosis and treatment of dermatomyositis has shown decreased risk of morbidity. Typically, upper esophageal dysphagia is expected as JDM is a myopathy of striated muscle. It is still unknown whether compromised intestinal motility is a by-product of calcinosis invading muscular layers inhibiting coordinated motility or an unusual manifestation in smooth muscle dysfunction due to underlying myopathy. It is important to understand this rare presentation as the best treatment has not been characterized well in literature. Our case demonstrates the importance of early and aggressive treatment for JDM with the goal of preventing late-stage manifestations of intestinal dysmotility and severe malnutrition.


Disclosures:


Patrick Tempera, DO¹, Skyelor Black, BS², Nour Parsa, MD². P1302 - Severe Malnutrition Secondary to Juvenile Dermatomyositis: A Rare Case Report, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.