Intestinal Pseudo-Obstruction - An Under-Recognized Presentation of Systemic Lupus Erythematosus

Intestinal pseudo-obstruction (IPO), characterized by obstruction without an identifiable anatomic cause, is a rare and poorly understood entity that may occur as a primary condition or secondary to other autoimmune disorders such as systemic lupus erythematosus (SLE). A12-year-old female child was brought with abdominal distension, vomiting, and fever for 15 days. Examination showed height and weight less than the third centile for age, tachypnea, tachycardia, and hypertension with severe abdominal distension, copious bilious aspirate, and very sluggish bowel sounds. Abdominal X-ray showed multiple air fluid levels. Ultrasound abdomen and unenhanced computed tomography (CT) scan revealed thickened dilated bowel loops, ascites, and pleural effusion. In view of multisystem nature of the disease, Koch's abdomen or autoimmune disease was suspected and emergency laparotomy procedure was deferred. She was evaluated and diagnosed to have SLE with lupus nephritis class V as per the International Society of Nephrology/Royal Pathology Society. She was managed conservatively with nasogastric decompression, immunosuppressive therapy and supportive hemodialysis.