Rescue Vedolizumab Therapy for a Rare Case of Complicated Severe Ulcerative Colitis: A Case Report and Literature Review

Background: Ulcerative colitis (UC) is a chronic inflammatory bowel disease associated with extraintestinal manifestations, including primary sclerosing cholangitis (PSC). Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that rarely coexists with UC or PSC. The concurrent occurrence of UC, PSC, and SLE in a single individual represents a unique diagnostic and therapeutic challenge. Vedolizumab, a gut-selective biologic agent, is effective for managing UC; however, its utility in patients presenting with this triad of conditions has not yet been explored. Case summary: A 32-year-old man presented with a 10-year history of recurrent upper abdominal pain, frequently accompanied by high-grade fever, along with recent onset of jaundice, diarrhea, hematochezia, and chronic rashes. Diagnostic evaluation confirmed PSC, SLE, and severe UC. During hospitalization, the patient also developed bacteremia. Initial management of UC with mesalazine and immunosuppressants (azathioprine followed by cyclosporine) resulted in limited clinical improvement. Vedolizumab was subsequently initiated, resulting in marked clinical improvements and near-complete endoscopic remission of UC. PSC and SLE remained clinically stable with ongoing therapies; however, the patient is currently awaiting liver transplantation for PSC. Conclusions: This case highlights the potential utility of vedolizumab in the treatment of UC in patients with concurrent PSC and SLE.