A 29-year-old female presented with recurrent, intermittent, non-localising abdominal pain. The pain was frequent, but not always, associated with eating and coincided with subjective fever, nausea, vomiting, early satiety and fatigue. Over the course of a year, weight loss (9 kg) and food avoidance were observed without diarrhoea, blood per rectum or melena. While otherwise healthy, the patient suffered from these painful attacks lasting several hours with increasing frequency and duration.
Other pertinent medical conditions included childhood asthma in remission, allergic rhinitis, hypertension, a positive purified protein derivative that had been treated with isoniazid, and normocytic anaemia attributed to β-thalassemia trait. The patient was a non-smoker, who did not drink excessive alcohol and was neither obese nor pregnant. She had a previous cholecystectomy for presumed biliary colic within a year prior to this presentation.
Physical examination revealed an abdomen that was soft, non-distended, with no masses palpable and diffuse tenderness. Abnormal laboratory assessments included an elevated erythrocyte sedimentation rate of greater than 100 on two separate occasions.
Occult malignancy, vasculitis or chronic mesenteric ischaemia was considered at this juncture. To evaluate further, a CT scan and an endoscopy of the abdomen were obtained. The patient had a normal colonoscopy and barium enema. Upper endoscopy was grossly normal; however, histopathology suggested Barrett's oesophagitis. The patient was treated with omeprazole without clinical improvement. A CT scan of the abdomen revealed non-specific inflammatory changes within the mesentery and small bowel thickening (). A subsequent CT scan showed interval progression of inflammation in addition to two peripheral portal vein thromboses in the left and right hepatic lobe. Abdominal, mesenteric, bilateral renal and ileocolic arteriograms showed no vasculitis or renal arterial stenosis. Her hypercoagulable investigation revealed heterozygosity for factor V Leiden and a slightly decreased antithrombin III (ATIII) level. The patient began anticoagulation with warfarin.
Figure 1 Inflammatory radiographic changes improve after initiation of infliximab (A) Three transverse CT images show extensive small bowel wall thickening (*) in multiple loops of bowel. Diffuse infiltration and stranding of adjacent mesentery (solid arrows) (more ...)
Despite anticoagulation and resolution of the thrombosis, the patient's symptoms did not improve. Consequently, the patient was hospitalised for an exploratory laparotomy. Histopathology of the mesentery and omentum showed chronic inflammation and fibrosis consistent with sclerosing mesenteritis (). Cultures of the peritoneal fluid grew two organisms, Morganella morganii and Enterococcus faecalis, for which the patient was treated with impenem (1 g every 8 h). After antibiotic completion, the patient was treated with prednisone (40 mg/day), azathioprine (75 mg/day) and oxycontin (10–20 mg/day).
(A) Mesenteric and (B) omental biopsy show fibrosis with chronic inflammation. Differential diagnosis includes infection versus sclerosing mesenteritis.
Despite immunosuppression, the patient was hospitalised eight times over the next year for severe abdominal pain. The frequency of hospitalisations increased as the patient's steroid doses were reduced, thus rendering her steroid and narcotic dependent. CT scans showed worsening inflammation, fluid in the midabdominal and pelvic regions and wall thickening and inflammatory changes of the jejunum. Inflammatory markers that correlated with the patient's abdominal symptom included a reactive leukocytosis (11.3–26.5; reference range 4.5–11×109/l), elevated erythrocyte sedimentation rate (26–61; reference range <20 mm/h) and anaemia (haematocrit 26–35.9; reference range 36–46%) that was independent of her known β-thalassemia trait (). High-dose prednisone (1–0.5 mg/kg) became necessary to control the patient's symptoms despite additional azathioprine (100 mg) and sulfasalazine (1000 mg). The patient then developed severe intermittent low-back pain. The musculoskeletal exam was initially normal, but radiographs revealed periarticular sclerosis at the sacroiliac joints and sacroiliitis ().
Figure 3 Markers of disease and treatment course with prednisone and infliximab. Infliximab infusions (arrows) were given at 5 mg/kg intravenous. Separate prednisone tapers were initiated during disease course, the first starting at 40 mg four times a day and (more ...)
Anteroposterior (left) and cephalically angulated (right) projections of the sacroiliac joints demonstrate sclerosis and subchondral erosions along the lateral portions of both sacroiliac joints.