S1930 Crohn’s Disease in a Patient With Fibrodysplasia Ossificans Progressiva

Abstract

Introduction: Fibrodysplasia ossificans progressiva is a rare disease (approximately 800 known cases) characterized by inflammatory soft tissue flares resulting in heterotopic ossification in skeletal muscles, fascia, and tendons with gradual restriction of movement due to joint ankyloses (Fig. 1). Flares are precipitated by minor trauma, over-exertion, intramuscular injections, and viral illness. In contrast to fibrodysplasia ossificans progressiva, inflammatory bowel disease is a relatively common condition and can go undiagnosed for years if it presents indolently. To our knowledge, there are no previous cases of coexisting Crohn’s disease and fibrodysplasia ossificans progressiva reported in the literature. Case description/methods: A 22-year-old male with history of fibrodysplasia ossificans progressiva presented with intermittent fevers and 20 pound weight loss over the preceding 6 months. He had no localizing symptoms other than mild cough and 2-4 loose stools daily without blood. Labs were remarkable for CRP elevated to 82 mg/L. Imaging revealed wall thickening and mural hyperenhancement of the distal ileum (Fig. 2). Extensive evaluation for an infectious etiology was negative. Colonoscopy revealed serpiginous ulcerations in the terminal ileum. Biopsies revealed active chronic inflammation and ulceration without granulomas. The history, radiography and histology were all consistent with Crohn’s disease. Discussion: This is the first case report of a patient with coexisting fibrodysplasia ossificans dysplasia and Crohn’s disease. Fibrodysplasia ossificans progressiva raises challenges in IBD management as many available therapies require IV or injectable administration. While subcutaneous and IV therapies can be administered to patients, care should be taken to avoid accidental IM administration or repeated misplaced IV’s as this may result in heterotopic ossification. Given these challenges, we prescribed oral methotrexate for initial treatment. If methotrexate fails, budesonide, azathioprine or perhaps off label upadacitinib are alternatives. For disease monitoring, colonoscopy is challenging because of anesthesia-related airway limitations. We plan to utilize fecal calprotectin and either capsule endoscopy or MR enterography to avoid the risks of repeat colonoscopy. It remains to be seen how the inflammatory milieu of Crohn’s disease impacts the course of fibrodysplasia ossificans progressiva, or if Crohn’s treatments could reduce the risk of future flares.Figure 1.: Heterotopic ossification and scoliosis on chest xray.Figure 2.: Ileal inflammation on MR enterography.