S4852 Ustekinumab in the Treatment of Intestinal Manifestations of Behcet's Disease

Introduction: Behcet’s disease (BD) is a chronic inflammatory disorder that affects multiple organ systems. Intestinal involvement occurs in 3%-60% of cases. The mainstay of treatment includes glucocorticoids, 5-aminosalicylic acids, immunomodulators, thiopurines, and biologics. Treatment recommendations for refractory cases reveals limited studies requiring a novel treatment plan. Case Description/Methods: Patient is a 50-year-old woman diagnosed with BD complicated by ileocolitis. She reports a history of recurrent oral ulcers in adolescence. She developed a clinically significant painful genital ulcer prior to diagnosis. Biopsy of the lesion showed vulvular neutrophilic dermatoses which prompted initiation of prednisone and cyclosporine. The patient developed hypertension on this regimen and transitioned to azathioprine. Development of nausea and vomiting limited dose escalation of azathioprine. She was switched to adalimumab, colchicine and hydroxychloroquine by rheumatology. On this regimen the patient developed BD colitis noted on computed tomography and severe oral ulcers requiring hospitalization. Her regimen transitioned to sulfasalazine, apremilast, and steroid taper. The medical team attempted methotrexate to prevent chronic glucocorticoid use, but the patient self-discontinued this medication due to gastrointestinal intolerance. This resulted in an emergency room visit due to severe left lower quadrant pain. Computed tomography scan showed a large ulcer in the ileum mimicking appendicitis. Severe BD flare was confirmed with colonoscopy. The patient was placed on intravenous glucocorticoids for flare management and initiated Ustekinumab. Maintenance dosing interval decreased to every 4 weeks with improvement in symptoms. Magnetic Resonance Imaging Enterography showed no evidence of ulceration or bowel inflammation. She remains in clinical remission on Ustekinumab, apremilast, and sulfasalazine. Discussion: A group of patients with intestinal BD experience disease refractory to mainstay therapy. Currently, no randomized controlled trials have evaluated the optimal strategy for their treatment. This case demonstrates that, in patients with intestinal BD, Ustekinumab appears to be a potentially effective treatment. It represents a promising treatment option for intestinal BD, and further studies are needed.