P3225 - Chronic Intestinal Pseudo-Obstruction: A Debilitating Complication of Scleroderma

Prekchha Jha, MBBS¹, Nadine S. Kassab, MD², Samiksha Pandey, MBBS, MSc³, Ihab Kassab, MD¹, Naveen Reddy, MD^1
1Corewell Health, Royal Oak, MI; ²Trinity Health Ann Arbor Hospital, Ypsilanti, MI; ³Corewell Health William Beaumont University Hospital, Berkley, MI

Introduction: Chronic Intestinal Pseudo-Obstruction (CIPO) is a rare, potentially disabling disorder affecting the peristalsis of gastrointestinal tract. It is a significant cause of dysmotility in systemic sclerosis. Here we present a case of abdominal symptoms indicative of CIPO. Despite interventions, her condition remained challenging, emphasizing the need for improved therapeutic approaches.

Case Description/Methods: A 62-year-old female with past medical history of chronic pancreatitis and untreated systemic sclerosis, presented to the emergency department for 2-week history of abdominal pain and progressively worsening abdominal distention. Her vitals were notable for hypotension and Computed tomography scan was concerning for distal small bowel obstruction along with pneumoperitoneum with pneumatosis throughout the small bowel concerning for ischemic bowel/perforation. She underwent an urgent exploratory laparotomy with colonic decompression. Intraoperative observations revealed extensive intestinal distension from the stomach to the colon, marked by minimal peristalsis, and notably, no evidence of visceral perforation or ischemia.




Postoperatively, Xray of the abdomen revealed persistently dilated bowel for which she required intermittent nasogastric tube decompression. Her symptoms were attributed to CIPO due to severe hypomotility in setting of advanced scleroderma. Despite trials with prokinetic agents such as erythromycin and Reglan, minimal improvement was observed. Rifaximin was added due to concerns of Small Intestinal Bacterial Overgrowth. Although there was slight improvement with pyridostigmine, she remained unable to tolerate a liquid diet, necessitating total parental nutrition. Her symptoms gradually improved, and she was discharged on Total Parental Nutrition (TPN). The patient is now being considered for Scleroderma Trial with possible initiation of Intravenous Immunoglobulin but unfortunately, she continues to be on TPN.


Discussion: CIPO stands as a rare complication of scleroderma, posing a significant medical challenge in treatment. Presently, there are no Disease Modifying Antirheumatic Drugs tailored for the gastrointestinal complications of scleroderma. Despite advancements in understanding, treatment remains largely symptomatic, contributing to heightened morbidity. Further research is crucial to develop targeted therapies to improve the quality of life in these patients.



Disclosures:


Prekchha Jha, MBBS¹, Nadine S. Kassab, MD², Samiksha Pandey, MBBS, MSc³, Ihab Kassab, MD¹, Naveen Reddy, MD¹. P3225 - Chronic Intestinal Pseudo-Obstruction: A Debilitating Complication of Scleroderma, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.