Clinical Analysis of 61 Systemic Lupus Erythematosus Patients With Intestinal Pseudo-Obstruction and/or Ureterohydronephrosis

Abstract

The objective of this article is to investigate the clinical features of intestinal pseudo-obstruction (IPO) and/or ureterohydronephrosis in systemic lupus erythematosus (SLE).Sixty-one SLE patients with IPO and/or ureterohydronephrosis were analyzed retrospectively. A total of 183 cases were randomly selected as controls from 3840 SLE inpatients without IPO and ureterohydronephrosis during the same period. Patients were assigned to 1 of the 3 groups (SLE with IPO and ureterohydronephrosis, SLE with IPO, and SLE with ureterohydronephrosis). The clinical characteristics, treatments, and prognosis were compared between the 3 groups.There were 57 females and 4 males, with a mean age of 32.0 years. IPO was the initial manifestation of SLE in 49.1% of the cases, whereas ureterohydronephrosis in 32.5%. All patients were initially treated with a high-dose steroid. Thirty-one of these patients (50.8%) also received intravenous methylprednisolone pulse therapy. Two patients died of bowel perforation and lupus encephalopathy, and the other 59 patients (96.7%) achieved remission after treatment. The incidences of fever, glomerulonephritis, nervous system involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and anti-SSA antibody positivity were significantly higher in patients with IPO and/or ureterohydronephrosis than in the control group (without IPO and ureterohydronephrosis). Also, patients with IPO and/or ureterohydronephrosis had higher SLE Disease Activity Index scores than control patients. Compared with SLE patients with IPO, the patients with IPO and ureterohydronephrosis had a significantly higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis, whereas the patients with ureterohydronephrosis had less mucocutaneous involvement and serositis. Eight of the 47 IPO patients who initially responded well to immunotherapy relapsed; however, all responded well to retreatment with adequate immunotherapy. Of these 8 patients, 4 relapsed following poor compliance and self-discontinuation of steroid or immunosuppressant therapy. The rate of poor compliance with immunotherapy and the number of organ systems involved in patients in the recurrent IPO group were significantly higher than those in the nonrecurrent IPO group.IPO and ureterohydronephrosis are severe complications of SLE. As patients usually respond readily to early optimal steroid treatment, early diagnosis and timely initiation of glucocorticoid are important to relieve symptoms, prevent complications, and improve prognosis.

A case with hydroureteronephrosis accompanied by Lupus Mesenteric Vasculitis

“…On the other hand, co-existence of intestinal pseudo-obstruction and hydroureteronephrosis has been previously reported in SLE patients [4]. In this case report, we describe a patient with SLE who presented with acute abdominal pain and who was subsequently found to have lupus mesenteric vasculitis in association with hydroureteronephrosis.…”

“…Initial step in management requires excluding other causes of abdominal pain including acute gastroenteritis, peptic ulceration, acute pancreatitis, and peritonitis. LMV is characterized by immune complex deposition, complement activation with subsequent submucosal edema, and leukocytoclastic vasculitis involving mesenteric vessels and thrombus formation [4]. In the early stages of LMV, high-dose steroids are generally fairly effective and complications such as intestinal ischemia, necrosis, or perforation may be prevented by medical treatment.…”

Complications of Systemic Lupus Erythematosus in the Emergency Department

“…Intestinal pseudo-obstruction typically responds well to corticosteroids and other immunosuppressant therapies. 26…”

Organ involvement other than lupus nephritis in childhood-onset systemic lupus erythematosus

“…22 There are no specific autoantibodies associated with IPO, but a high prevalence of anti-SSA antibodies has been reported. 34,35 IPO can be the presenting feature of SLE and frequently occurs in the setting of active disease, but can also occur with low SLE activity. 35 cSLE and aSLE patients with IPO and/or ureterohydronephrosis must be regarded as having active SLE independent of the disease activity scoring and given early aggressive treatment with high-dose corticosteroids with or without other immunosuppressive agents, as well as oral broad spectrum antibiotics, promotility drugs such as octreotide, and bowel rest.…”

“…34,35 IPO can be the presenting feature of SLE and frequently occurs in the setting of active disease, but can also occur with low SLE activity. 35 cSLE and aSLE patients with IPO and/or ureterohydronephrosis must be regarded as having active SLE independent of the disease activity scoring and given early aggressive treatment with high-dose corticosteroids with or without other immunosuppressive agents, as well as oral broad spectrum antibiotics, promotility drugs such as octreotide, and bowel rest. 26,36 Delay in diagnosis may result in life-threatening complications such as bowel necrosis, bowel perforation and acute renal failure.…”