Chronic Intestinal Pseudo-obstruction in the Setting of Inactive Systemic Lupus Erythematosus

Abstract

Chronic intestinal pseudo-obstruction (CIPO) is a rare disorder of intestinal hypomobility, which presents a similar fashion to a bowel obstruction, but without evidence of anatomic obstruction. We present a case of CIPO associated with inactive systemic lupus erythematosus (SLE).A 42 year-old woman with a history of SLE presented with obstipation, with episodic nausea, vomiting, and abdominal pain. No prior history of abdominal surgery. CT abdomen & pelvis did not reveal any anatomic obstruction. Colonoscopy showed no masses. Impaired intestinal motility was confirmed with scintigraphy, consistent with CIPO. Anti-rho antibody was positive. She was treated with laxatives, enemas, and pro-motility agents. Since being diagnosed with SLE patient encountered several complications, including pulmonary hemorrhage, hemolytic anemia, nephritis, and retinitis from hydroxychloroquine. Aside from bowel disturbances, the patient remained asymptomatic between lupus flares. Over several years, the patient developed 6 episodes of acute colonic pseudo-obstruction, treated with intravenous methylprednisolone. Exploratory laparotomy showed a large, dilated colon with fecal impaction, without any anatomic cause of obstruction. A subtotal colectomy and end ileostomy were performed. Pathology showed mucosal ulceration with transmural fibrosis, and pericolic lymph nodes with reactive follicular hyperplasia. She did well initially, but then required several ostomy revisions for pseudoobstruction. Ultimately, her stoma prolapsed, requiring decompression.The etiology of CIPO is unknown, but may involve vasculitis. Several pathologic changes may be observed, including fibrosis. CIPO is often idiopathic, but may occur due to an underlying condition, such as lupus. CIPO usually occurs in the setting of active lupus. No laboratory tests are specific for CIPO; however, 83% of cases have anti-Rho antibodies.To our knowledge, this represents the second reported incidence of intestinal pseudo-obstruction associated with inactive systemic lupus erythematosus. There are less than 35 cases of CIPO associated with SLE published in English. CIPO is a rare and life-threatening complication of lupus. Awareness of the condition may facilitate early recognition and appropriate treatment, which includes intravenous glucocorticoids, immunosuppressants, and promotility agents. When possible, surgery should be avoided, as the condition tends to recur.Figure. X: ray abdomen showing diffusely dilated small bowel loops.Figure: Trichrome stain, showing transmural fibrosis.Figure: Elastic stain to show intimal fibroplasia and luminal stenosis.