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.