Chronic Intestinal Pseudo-Obstruction in Systemic Sclerosis: An Uncommon Presentation

Abstract

Chronic intestinal pseudo-obstruction (CIPO) is an uncommon disorder characterized by the presence of bowel dilatation and abnormal motility. It is an important cause of chronic intestinal failure in patients with systemic sclerosis (SSc). Although intestinal pseudo-obstruction is an infrequent reason for hospitalization in these patients, it has been correlated with high in-hospital mortality compared to SSc patients hospitalized for other reasons as well as patients with intestinal pseudo-obstruction arising from other causes. Patients present with signs and symptoms of mechanical bowel obstruction, such as nausea, vomiting, constipation, abdominal pain, and abdominal distension, in the absence of an anatomic lesion blocking the movement of intestinal contents. Despite breakthroughs in our understanding of this disorder, these patients continue to be treated largely with organ-based symptomatic therapy. Unfortunately, despite treatment, they often experience decreased quality of life and impairment in their everyday lives. Here, we present an interesting case of an individual with SSc for many years who presented with signs and symptoms of CIPO.