Severe intestinal involvement in Wegener's granulomatosis: report of two cases and review of the literature

The clinical and pathological manifestations of severe intestinalinvolvement in Wegener's granulomatosis were studied by a review of theliterature and reports of two patients. Altogether, six cases, two femalesand four males, were studied. One patient developed two episodes of bowelmanifestations necessitating immediate surgical interventions. The averageage at onset of intestinal symptoms was 43.3 yr (26-55 yr) and, in allcases, the first signs of such manifestations developed within the first 2yr of disease. Prior to the onset of intestinal symptoms, immunosuppressivetherapy was administered in six of seven instances. Acute abdominal painwith signs of peritonitis or distention only constituted the main clinicalpicture in six of the seven events. The last episode was manifestedclinically with profuse diarrhoea with blood and mucus. Of the seveninstances of severe intestinal manifestations, the small bowel was involvedin two, the large bowel in three, and both the small and large bowel wereaffected in two episodes. Histological evidence of vasculitis in the bowelwas demonstrated in three of the seven biopsy specimens, while in four,ischaemia, inflammation and ulceration were the pathological findings.Intestinal perforation was seen four times and surgery was performed in sixof seven episodes. Severe intestinal involvement is rare in Wegener'sgranulomatosis. The initial bowel manifestations occur within the first 2yr of disease, and affect both the large and small bowel. Histologically,vasculitis, ischaemia, inflammation and ulceration are the prevailingfindings. Death due to intestinal catastrophy occurred in one of the sixpatients reported. Most likely, the manifestations are associated with thedisease process rather than related to the use of immunosuppressive agents.