Intestinal Pseudo-Obstruction as a Presenting Manifestation of Systemic Lupus Erythematosus: Case Report and Review of the Literature

Hien Nguyen, MD; Niharika Khanna, MD

South Med J. 2004;97(2) 

In This Article

Abstract and Case Report

A case of intestinal pseudo-obstruction as a presenting manifestation of systemic lupus erythematosus is presented. Systemic lupus erythematosus is an autoimmune disease with a wide spectrum of unpredictable multisystemic presentations. A case of systemic lupus erythematosus that was diagnosed in a patient whose initial presentation for the disease was acute renal failure, hydronephrosis, intestinal pseudo-obstruction, and false-positive syphilis serologies is presented. There have been 18 other cases of intestinal pseudo-obstruction associated with systemic lupus erythematosus to date in the English literature. A variety of gastrointestinal syndromes have been increasingly recognized as possible manifestations of systemic lupus erythematosus, and this case illustrates in particular that intestinal pseudo-obstruction may be a presenting manifestation of this disorder.

A 35-year-old woman with hypertension and a history of IV drug use presented with 3-day history of crampy, nonradiating, midepigastric, abdominal pain; and distension, anorexia, vomiting, and diarrhea. There was no fever, hematemesis, or hematochezia. She also complained of mild swelling and pain in her knees. She denied chest pain, shortness of breath, headache, or rashes. There was no history of unusual travel or food ingestions, sick contacts, or antibiotic use. She had a history of IV cocaine and heroin abuse and reported that her last use was 1 year before admission. She had been diagnosed 3 years previously with syphilis, for which she had received penicillin therapy. There was a remote surgical history of femoral hernia repair and prior obstetric history significant for four first-trimester miscarriages and no livebirths. There was a strong family history of systemic lupus erythematosus in her mother and aunt. Medications at admission included Norvasc.

The cardiopulmonary examination was significant for a 2/6 systolic ejection murmur and fine basilar crackles. Abdominal examination revealed hypoactive bowel sounds, distended abdomen with diffuse tenderness, and tympany without peritoneal signs. Rectal examination demonstrated heme-negative brown stool without masses.

The musculoskeletal examination showed mild bilateral knee effusion with preserved range of motion. The dermatologic examination was significant for seborrheic dermatitis, nonscarring alopecia, and malar rash, without physical signs of scleroderma including sclerodactyly or telangiectasias.

Complete blood count was significant for a microcytic anemia with hematocrit of 25.1/µl and mean corpuscular volume of 72.5. Anemia workup was consistent with a combined iron deficiency anemia and Coombs-positive hemolytic anemia. Serum chemistries revealed acute renal failure with blood urea nitrogen of 53 mg/dl and creatinine of 2 mg/dl. Liver function tests were normal except for albumin of 2.6 mg/dl, and amylase and lipase levels were normal. Abdominal flat and upright films demonstrated multiple air fluid levels and distended loops of small bowel. The urinalysis demonstrated active urinary sediment with 4+ protein and few red blood cell casts, but was negative for illicit drugs. Renal ultrasound showed echogenic kidneys consistent with medical renal disease and mild bilateral hydronephrosis, and 24-hour urine studies demonstrated non-nephrotic range proteinuria with a total of 850 mg protein and creatinine clearance of 50 ml/min. Serologies for connective tissue disease revealed positive antinuclear antibody titer of 1:640 with speckled pattern. Smith antibody was positive but rheumatoid factor, ribonucleotide protein, Ro, La, double-stranded DNA, anti-proliferating cell nuclear antigen, SCL-70, and anticentromeric antibody were all negative. Complement levels were decreased at 40 mg/dl (C3) and 10 mg/dl (C4), and viral hepatitis and human immunodeficiency virus serologies were negative. Lupus anticoagulant and anticardiolipin antibodies were both positive and consistent with her history of multiple early miscarriages. A retrospective chart review showed that the patient had positive rapid plasma reagin serologies for which she had received penicillin injections but negative fluorescein treponema antibody, consistent with false-positive syphilis testing. Repeat antitreponemal tests during this admission confirmed false-positive syphilis serology.

The patient was admitted. Bowel rest was instituted with placement of a nasogastric tube, and hydration was initiated with IV fluids. In consultation with the gastroenterology department, a small bowel contrast study and colonoscopy were performed 1 week into admission, as her obstruction improved, ruling out mechanical obstruction in the small and large intestines. A renal biopsy was performed, demonstrating mild mesangial proliferative changes consistent with Class II lupus nephritis. The patient was initiated on high-dose IV prednisone and erythromycin and weaned to oral feeds by Week 2 of admission. The patient's creatinine stabilized to 1.0 mg/dl with steroidal therapy, and the patient was discharged on oral prednisone 2 mg/kg/d for 2 weeks. At 6-week follow-up, on a maintenance dose of prednisone 10 mg/d, there was complete resolution of the intestinal pseudo-obstruction and hydronephrosis, without recurrence of nephritis.

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