Severe Intestinal Involvement in Wegener's Granulomatosis: Report of Two Cases and Review of the Literature

The clinical and pathological manifestations of severe intestinal involvement in Wegener's granulomatosis were studied by a review of the literature and reports of two patients. Altogether, six cases, two females and four males, were studied. One patient developed two episodes of bowel manifestations necessitating immediate surgical interventions. The average age at onset of intestinal symptoms was 43.3 yr (26-55 yr) and, in all cases, the first signs of such manifestations developed within the first 2 yr of disease. Prior to the onset of intestinal symptoms, immunosuppressive therapy was administered in six of seven instances. Acute abdominal pain with signs of peritonitis or distention only constituted the main clinical picture in six of the seven events. The last episode was manifested clinically with profuse diarrhoea with blood and mucus. Of the seven instances of severe intestinal manifestations, the small bowel was involved in two, the large bowel in three, and both the small and large bowel were affected in two episodes. Histological evidence of vasculitis in the bowel was 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 six of seven episodes. Severe intestinal involvement is rare in Wegener's granulomatosis. The initial bowel manifestations occur within the first 2 yr of disease, and affect both the large and small bowel. Histologically, vasculitis, ischaemia, inflammation and ulceration are the prevailing findings. Death due to intestinal catastrophy occurred in one of the six patients reported. Most likely, the manifestations are associated with the disease process rather than related to the use of immunosuppressive agents.