Discussion:
Gastrointestinal involvement is not common among patients presenting SLE [1]. The clinical manifestations may essentially include lupus mesenteric vasculitis, intestinal pseudo-obstruction, protein losing enteropathy, pancreatitis and hepatobiliary manifestations [1]. Intestinal pseudo-obstruction is a gut motility disorder and a rare condition when associated to SLE.
Pathogenesis of SLE-related IPO remains unclear. It can probably be due to intestinal vasculitis affecting visceral smooth muscles or to autoantibodies targeting smooth muscle with immune complex deposition on the muscle and/or nerve explaining gut dismotility disorder during SLE [3].
The diagnosis of IPO is made based on clinical signs, air-fluid levels and bowel dilatation, with or without thickened bowel wall in radiologi­cal imaging. Esophagogastroduodenoscopy, colonoscopy, and abdominal CT scan are needed to exclude mechanical ob­struction [4]. Intestinal manometry may be needed to exclude systemic sclerosis or Hirschprung’s disease [5].
Diagnosis might be challenging especially if IPO is the first manifestation of SLE [3]. Misdiagnosis rate is very high, up to 78% [2]. However it is important to promptly recognize this rare condition since misdiagnosis can lead to an unnecessary surgical intervention [3].
Ureterohydronephrosis, anti-U1 RNP antibodies, peritonitis and low C3 level were identified as being independent predictors of IPO in SLE [6]. Thus, authors recommend regular abdominal X-ray examinations in patients with these predictors. In our case, hydronephrosis was noticed at first presentation.
Supportive treatments of IPO include parenteral nutrition, prokinetics (neostigmine, erythromycin) and antibiotics against bacterial overgrowth [7]. In addition, specific treatment of SLE-related IPO is based on corticosteroid therapy +/- associated with immunosuppressants (cyclophosphamide / azathioprine / tacrolimus) [3,7]. In a review of the literature published by Wang et al, 27 patients received corticosteroids as initial therapy. A good response was obtained in 81% of the cases [3]. However, none of the patients who underwent surgical intervention achieved long-term improvement [3,7]. In addition to ineffectiveness, unnecessary surgical intervention is responsible of morbi-mortality. However, urgent surgery must be indicated in acute complications of IPO such as intestinal perforation or ischemia. Regarding prognostic, IPO-related SLE can cause significant morbidity and mortality if not rapidly diagnosed and treated. In another review of the literature [7], mortality rate was of 6.99% (10/143). Nephrotic syndrome, ureterohydronephrosis and megacholedochus are independent poor prognostic factors in SLE-related IPO [2]. In this report, although the patient had hydronephrosis, she presented favorable outcome.
It is important to timely initiate medical treatment, otherwise the smooth muscle layer can progress to fibrosis, become atrophic and no longer reversible [8].
We reported a case of IPO presenting as the initial manifestation of systemic lupus erythematosus. The diagnosis of IPO was based on clinical signs, X-ray, CT scan and endoscopy. The diagnosis of SLE was made upon clinical, biological criteria and immunological findings. The patient had a spectacular response to steroids. Treatment with corticosteroids alone was sufficient and the patient did not require other immunosuppressive therapy.
In conclusion, SLE-related IPO is rare and may be a challenging diagnosis especially if the underlying disease is not yet established. Systemic disease should be always considered when managing unexplained IPO, as medical treatment based on corticosteroids +/- immunosuppressive therapy is very efficient.