Intestinal pseudo‐obstruction in systemic lupus erythematosus

Abstract

In order to document intestinal pseudo-obstruction (IPO) as a recently recognized manifestation of systemic lupus erythematosus (SLE), we report the case or a woman with SLE who presented with IPO and we review 21 other previously reported cases from an English literature search. In 41% of the cases, IPO was the initial manifestation of their underlying lupus. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for an apparent association with an urinary tract involvement (ureterohydronephrosis and interstitial cystitis). The pathogenic mechanism of this complication is not fully understood, but seems to be heterogeneous. IPO responded readily to high dose steroid therapy in all patients, but in some cases this complication evolved regardless of the underlying disease activity. A high level of awareness of this complication is needed to avoid unnecessary surgical intervention.

Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

“…The pathophysiology of IPO secondary to SLE remains unclear [3], though several hypotheses have been proposed [1,4,[6][7][8]. The high association between IPO and ureterohydronephrosis suggests a smooth muscle dysmotility due to a primary neuropathy or myopathy, or secondary to either an immune complexmediated vasculitis or common autoantibody against the smooth muscles [4] Generalized vasculitis involving inXammatory Wbrinoid deposits or smooth muscle muscularis propria dysmotility may also aVect the smooth muscle of the intestine and lead to small bowel obstruction.…”

A case with hydroureteronephrosis accompanied by Lupus Mesenteric Vasculitis

“…Pathophysiological mechanisms underlying the development of IPO in SLE have not been clearly elucidated, although proposed mechanisms include development of visceral smooth muscle injury and dysmotility subsequent to immune-complex mediated vasculitis. The smooth muscle dysfunction may be diffuse and may involve urinary and biliary systems as well [8][9][10]. Mesenteric ischemia due to LMV may be associated with a number of findings in abdominal CT including focal or diffuse intestinal wall thickening, enlarged intestinal segments, abnormal contrast enhancement in the intestines (target or double halo sign), mesenteric edema, engorgement of the vascular structures penetrating the intestinal wall, and ascites.…”

Gastrointestinal and Hepatic Manifestations of Systemic Lupus Erythematosus

“…Motility studies show esophageal and intestinal hypomotility, decreased lower esophageal sphincter pressure, and delayed gastric emptying. 40,41 IPO is commonly associated with urinary tract involvement with ureterohydronephrosis in two-thirds of patients, suggesting a smooth muscle dysmotility due to detrusor muscle spasm and secondary vesiculoureteric reflux or to fibrosis of the ureterovesicular junction. 40 Onethird of patients have interstitial cystitis, a well-recognized complication of SLE, resulting from immune complex deposition in the vessel walls of the bladder.…”

“…38,39 Small bowel is more frequently involved than large bowel. 40 Patients present with abdominal pain, nausea and vomiting, abdominal distension, and constipation. They may have dysuria without a positive bacterial culture.…”

“…Radiologic examinations show dilated, fluid-filled bowel loops with thickened bowel wall and multiple air-fluid levels. 40 Other causes of intestinal obstruction must be ruled out. Motility studies show esophageal and intestinal hypomotility, decreased lower esophageal sphincter pressure, and delayed gastric emptying.…”