Brief Report

Intestinal pseudo-obstruction as the initial presentation of systemic lupus erythematosus: the need for enteroscopic evaluation

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple systemic manifestations. The disease itself typically causes only 2–30% of SLE-associated gastrointestinal conditions.

We 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.

Intestinal 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.

Intestinal pseudo-obstruction (IpsO) is considered a severe manifestation of systemic lupus erythematosus (SLE) characterized by clinical and radiological evidence of intestinal obstruction with no identifiable mechanical lesion. We performed a systematic review to document IpsO in SLE. Twenty-eight articles with 42 patients were included. The median age of onset of IpsO was 27.5 (10–57) years. The female to male ratio was 38:4. Twenty-two (52.4%) patients had IpsO as the initial presentation of their underlying lupus. Three (7.1%) patients manifested in inactive lupus. The duration of abdominal symptoms before admitted ranged from 3 days to 3 years, however most of the patients responded well to systemic corticosteroid or immunosuppressive treatment within 2 days to about 3 months. Concomitant ureterohydronephrosis was present in approximately three-fourths of the cases. More interestingly, 4 patients presented hepatobiliary dilatation without mechanical obstruction together with IPO and ureterohydronephrosis. In conclusion, IpsO is an uncommon but important manifestation of SLE. The finding of coexisting ureterohydronephrosis and hepatobiliary dilatation suggests that there may be generalized visceral muscle dysmotility. Early recognition of IpsO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.

La pseudo-obstruction intestinale (POI) est une manifestation rare, mais potentiellement grave du lupus érythémateux systémique (LES). Nous rapportons l’observation d’une jeune femme de 24 ans suivie pour un LES depuis deux ans et qui était hospitalisée pour douleurs abdominales, vomissements post-prandiaux précoces avec arrêt du transit et un météorisme abdominal à l’examen. La radiographie d’abdomen sans préparation montrait des niveaux hydro-aériques de type grêlique. Le scanner abdominal objectivait une dilatation de l’intestin grêle surtout iléale associée à un amincissement de la paroi sans obstacle décelable ainsi qu’une dilatation urétéropyélocalicielle bilatérale. L’évolution était favorable sous corticothérapie par voie générale. La POI associée au LES survient souvent au cours de l’évolution de la maladie, mais peut être inaugurale. Seul un diagnostic précoce permet d’éviter un geste chirurgical inapproprié puisque l’évolution est souvent favorable sous de corticoïdes ou immunosuppresseurs.

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.