An unusual case of intestinal pseudo-obstruction presenting in an adolescent with juvenile-onset systemic lupus erythematosus: A diagnostic challenge

Abstract

IntroductionSystemic lupus erythematosus (SLE) is an autoimmune disease with multiple systemic manifestations. The disease itself typically causes only 2–30% of SLE-associated gastrointestinal conditions. Case reportWe present the case of a 16-year-old male with history of SLE diagnosed 5 months prior to admission. Patient was non-compliant to medical treatment. He presented with 20 days of cough, mucopurulent and blood-tinged sputum, progressive shortness-of-breath and abdominal bloating. Patient was found to have multiple organ dysfunction due to an active lupus flare that developed during hospitalization, and required treatment with high doses of corticosteroids and close observation in an intensive care unit. Despite initial improvement of symptoms, he continued with abdominal pain, bloating, abolished bowel sounds and poor food intake. An abdominal X-ray showed signs of intestinal obstruction, establishing the diagnosis of intestinal pseudo-obstruction (IpsO). Patient then displayed a marked improvement of his gastrointestinal condition following treatment with 400 mg/kg/day of intravenous immunoglobulin (IVIgG) for 5 days. ConclusionIntestinal pseudo-obstruction is an unusual clinical manifestation of SLE and may represent a diagnostic challenge. We underscore the importance of a prompt and precise recognition of this condition, which is likely to have a positive impact on clinical outcomes. IpsO is caused by a non-mechanical obstructive bowel injury. Evidence points towards to the use of IgG and steroid for five days as the mainstay of therapy for patients with IpsO.