Intestinal Pseudo-Obstruction Secondary to Lupus Enteritis: A Rare but Significant Complication of Systemic Lupus Erythematosus (SLE)

¹Shaista Khadim, ¹Sallam Saki, ¹Ethar Malik, ¹Elsadeg Sharif

¹Al Jalila Children’s Specialty Hospital

Background:

Lupus enteritis, although rare, is one of the leading causes of systemic lupus erythematosus (SLE) related acute abdominal pain in patients of SLE. By definition, lupus enteritis is either vasculitis or inflammation of the small bowel. It can have a poor prognosis in adolescents and children leading to life-threatening complications if not managed timely. Its pathology is often hypothesised to be due to immune-complex deposition and complement activation leading to submucosal edema. A severe manifestation in SLE patients with acute abdominal pain and vomiting is intestinal pseudo-obstruction (IpsO) which presents similar to an intestinal obstruction without any isolated mechanical cause. Diagnosis is usually made based upon clinical and radiological evidence.

We present a case of a 13 year old girl who was recently diagnosed with SLE and was on tapering doses of steroids when she presented to us with severe abdominal pain, associated with nausea and bilious vomiting. Pertinent labs revealed elevated ESR, low C3 and C4, elevated CRP, hypoalbuminemia. She underwent a CT abdomen which revealed classic findings of lupus enteritis including dilated small bowel loops, target sign and comb sign causing intestinal pseudo-obstruction. Pleural effusion and ascites were also noted. A multidisciplinary team approach was adopted (including surgery, gastroenterology, rheumatology, nephrology, radiology) and a final diagnosis of intestinal pseudo-obstruction secondary to inflammatory enteritis was made. She was started on high dose prednisolone and mycophenolate mofetil, to which she improved significantly and was subsequently discharged after a stay of 25 days in the hospital. She was also diagnosed with lupus nephritis grade II during the stay and was discharged on lisinopril. A magnetic resonance enterography done prior to discharge revealed significant resolution of findings noted in the bowel with residual activity.