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Although abdominal complications are occasionally reported in the Churg-Strauss syndrome (CSS), bowel perforations, cholecystitis, eosinophilic peritonitis, and oophoritis are very unusual1–8 and normally resolve after immunosuppressive treatment. We report the case of a patient with CSS with these complications, which was fatal despite proper treatment.
A 64 year old woman with a 13 year history of urticaria, recurrent rhinitis, and asthma was admitted for abdominal pain. An increasing peripheral eosinophilia rising from 1% to 22% in the past five years was detected. Two years before hospital admission an extensive urticariform erythema developed. An abdominal ultrasonography performed during an asthmatic exacerbation when she had no abdominal pain disclosed a thick-walled gall bladder with no echogenic contents. An excised nasal polyp showed polypoid hyperplasia with many eosinophils.
Two and six weeks later she was readmitted owing to right upper quadrant pain. The leucocyte count was 1×1010/l with 34% eosinophils. Abdominal ultrasonography and computed tomography (CT) scan showed acalculous cholecystitis. A laparotomy disclosed a purulent peritoneal collection and enlarged inflamed gall bladder and right ovarium. A cholecystectomy and right anexectomy were performed.
One month after surgery she was readmitted with severe abdominal pain, diarrhoea, and fever. The leucocyte count was 4.49×109/l with 22% eosinophils, erythrocyte sedimentation rate (ESR, Westergren) 39 mm/1st h, rheumatoid factor (RF) 765 IU/ml (normal <80), and total IgE 769 IU/ml (normal <100), and serum urea and creatinine, complement C3 and C4, antinuclear antibody and antineutrophil cytoplasmic antibody values were normal or negative. The urine contained 300 mg/l proteins and the sediment 6–8 red cells/low power field, 3–5 leucocytes, and hyalin and hyalinogranular casts. An abdomen CT scan showed moderate ascites. The ascitic fluid was serofibrinous with a protein concentration of 55 g/l, a leucocyte count of 1.05×109/l with 44% eosinophils, and negative standard and Lowënstein cultures. A diagnosis of CSS was made after reviewing the previous gallbladder and ovarium histopathological specimens (fig 1) and considering the history of asthma, eosinophilia, and nasal polyposis.9
Oral methylprednisolone 60 mg/day and cyclophosphamide 100 mg/day were started, with initial clinical improvement. However, the abdominal pain recurred and the patient underwent a second laparotomy after three weeks of treatment. Peripheral blood leucocytes were 18.1×1010/l with 1% eosinophils. Blood urea, creatinine, and urinary sediment were normal, the ESR fell to 15 mm/1st h and the RF to 435 IU/ml. Purulent fluid in the peritoneal cavity and two perforations in the ileal wall were found. Bowel histology showed wall ulcerations, vascular thrombosis with fibrinoid necrosis, and eosinophilic infiltrates. Granulomas were not found. E coli grew from the peritoneal fluid. Intravenous metronidazole and gentamicin were started. Four days later a new perforation was suspected and a third laparotomy was done, showing a perforated necrotic small bowel plaque. A broad bowel resection was performed but the patient's evolution was complicated with high fever, ileus, and vomiting, and she died 48 hours later. A necropsy was not allowed.
Abdominal pain is reported in up to 29–59% of cases of CSS, although many times the cause is unknown. Gastric and colonic ulcers, intestinal fistulas, and small bowel perforations have been described,1–5,8 the last of these being responsible for up to 10% of the CSS deaths.3 Acalculous cholecystitis, although very rare, may be the first and sometimes the unique manifestation of the CSS.5,6 Its evolution is usually torpid, and sometimes only diagnosed at necropsy. Abdominal ultrasonography should be included in the routine screening of patients with CSS.
The right oophoritis was due to vasculitis, with an eosinophilic infiltrate suggestive of CSS (fig 1). As far as we know, this is the first reported case of CSS with confirmed ovarium involvement.
The ascitic fluid, rich in eosinophils, the eosinophilic infiltration of major omentum samples and the clinical evolution suggest that the peritoneal involvement was due to the CSS, an extremely rare complication of this disease. Eosinophilic peritonitis was suggested by Lanham owing to serosal involvement in the CSS,2 but has only been confirmed in one case so far.8
The poor response to steroids and cyclophosphamide is striking. Despite the reduction of the peripheral eosinophilia and ESR there was widespread eosinophilic bowel infiltration and vascular fibrinoid necrosis in the laparotomy samples. The evolution of the disease in our patient was catastrophic, especially as she had only one of the five Guillevin CSS mortality associated factors—namely, gastrointestinal involvement.10
In summary, CSS abdominal complications should be promptly suspected and treated. In addition, CSS ovarian involvement, although rare, should be included in the differential diagnosis of ovary vasculitis.