P3143 - Treatment of Refractory Lupus Intestinal Pseudo-Obstruction with the Euro-Lupus Protocol

Award: Presidential Poster Award

Caroline Wang, MD, Manavi Bhagwat, MD, Ram Al-Sabti, MBChB, Farnaz Shariati, MD
Rush University Medical Center, Chicago, IL

Introduction: Intestinal pseudo-obstruction (IPO) is a rare complication of lupus with a prevalence of approximately 2% in hospitalized patients. Current management consists of lupus-directed therapy including steroids with or without disease-modifying antirheumatic drugs (DMARDs). The Euro-lupus protocol, consisting of 6 cycles of low-dose cyclophosphamide, is classically used to treat severe lupus manifestations such as nephritis. In this report, we present a case of lupus IPO refractory to traditional DMARDs but which responded to the Euro-lupus protocol.

Case Description/Methods: A 28 year old female with lupus previously diagnosed with pancolitis and hydroureteronephrosis presented with persistent abdominal pain and distention, diarrhea, and high nasogastric (NG) tube output for which she had been initiated on total parenteral nutrition. CT imaging showed dilated bowel loops without mechanical obstruction and colonoscopy revealed multiple scattered ulcerations in the sigmoid, descending and transverse colon. Biopsies of these ulcers noted acute and chronic inflammation but were negative for viral inclusions such as CMV and HSV. Abdominal CT angiography returned negative for vascular abnormalities including vasculitis. Overall, her clinical picture was most consistent with lupus IPO. She received treatment with daily intravenous (IV) methylprednisolone transitioned to pulse dose steroids followed by a one-week course of IV azathioprine and one time dose of IV belimumab but remained refractory with persistent abdominal distention and high NG output. The patient then underwent a kidney biopsy which showed severe lupus nephritis. She was initiated on the Euro-lupus protocol for her severe multi-organ lupus in combination with IV methylprednisolone with significant clinical resolution.

Discussion: The Euro-lupus protocol, a modification of high-dose IV cyclophosphamide therapy, was originally developed to minimize gonadal toxicity associated with cyclophosphamides. It has documented success in treating lupus nephritis with a ten-year followup study demonstrating comparable efficacy to the standard cyclophosphamide regimen. Its role in treating other lupus manifestations, however, are limited, although one case reported success with treating lupus enteritis. To date, there are no reports of its use in the treatment of lupus IPO. Our case thus highlights its potential as a therapy in lupus IPO unresponsive to traditional therapies.


Disclosures:


Caroline Wang, MD, Manavi Bhagwat, MD, Ram Al-Sabti, MBChB, Farnaz Shariati, MD. P3143 - Treatment of Refractory Lupus Intestinal Pseudo-Obstruction with the Euro-Lupus Protocol, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.