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A retrospective study in China defines the prevalence and course of disorders of the intestines or ureter secondary to systemic lupus erythematosus.
Xu Na, Zhao J, Liu Jinjing, et al. Clinical Analysis of 61 Systemic Lupus Erythematosus Patients With Intestinal Pseudo-Obstruction and/or Ureterohydronephrosis: A Retrospective Observational Study. Medicine. 2015; 94(4)e419 doi: 10.1097/MD.0000000000000419
Intestinal pseudo-obstruction (IPO) and ureterohydronephrosis (UH) are rare but severe complications of systemic lupus erythematosus (SLE). They usually respond readily to early steroid treatment.
They are characterized by ineffective intestinal propulsion, with signs and symptoms of intestinal obstruction, but without obstruction.
Out of 3,910 SLE inpatients at Peking Union Medical College, Beijing, 61 had IPO, UH, or both. There were 57 females and 4 males.
Of the 61 patients, 32 had both IPO and UH, 21 had IPO alone, and 8 had UH alone.
For those who had IPO, it was the initial manifestationof SLE in half the patients. In a third of patients who had UH, that was the initial manifestation of SLE.
Before admission, patients were misdiagnosed with tuberculosis, infective enteritis, and acute appendicitis.
All patients were treated with high-dose steroids, and half had intravenous methylprednisone pulse therapy.
Two patients died of bowel perforation or lupus encephalopathy, but the other 59 achieved remission after treatment. Eight patients relapsed, but responded well to retreatment. Of those who relapsed, four were able to discontinue treatment.
The incidence of fever, glomerulonephritis, nervous sytem involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and positive anti-SSA antibody were significantly higher in patients with IPO, UH, or both than in a comparison group of randomly selected SLE patients. They also had higher SLE Disease Activity Index scores.
Patients with IPO and UH had a higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis than patients with IPO alone.
In SLE, IPO is often associated with UH and interstitial cystitis, and may involve the esophagus and gallbladder.
The underlying mechanism may involve primary myopathy, neuropathy, vasculitis, or antibodies directed against the visceral smooth muscles, causing muscular damage and dysmotility.
Six patients had biliary tract dilatation without mechanical obstruction, which also suggests smooth muscle dysmotility. Biliary dilatation can lead to cholecystitis, cholangitis and generalized sepsis, so physicians should be aware of this possibility and obtain appropriate imaging early.
Early diagnosis and treatment are crucial for the recovery of visceral peristaltic function. Patients show an excellent response to early steroid therapy. But a delay in treatment or inadequate steroid dose may lead to advanced and irreparable tissue destruction, including fibrosis and atrophy of the muscularis propria, resulting in failure to regain functional peristalsis.