Department of Nephrology, Pitié Salpétrière Hospital, Paris, France
Sir,
Wegener's granulomatosis (WG) has not been included in the differential diagnosis for ulcerative colitis or Crohn's disease. However, affection of the gut has been detected at autopsy in a significant number of patients [1] even in the absence of a WG relapse. We present a case in whom ileitis was the initial presentation of WG disease relapse.
Case.
The patient was a 36-year old man who presented in October 1991 with fatigue, weight loss, sinusitis, acute renal failure, and pulmonary infiltrates. Kidney biopsy revealed pauci-immune crescentic glomerulonephritis without necrotizing lesions or interstitial abnormalities. cANCA were positive (titre 250). WG was diagnosed and cyclophosphamide and corticosteroids administered for 18 months. At the end of treatment, cANCA were negative and remained so 5 years later.
In December 1999, the patient was admitted to our department because of acute abdominal pain with distension and diarrhoea without blood or mucus. C-reactive protein was 80 mg/l and moderate fever developed. Colonoscopy revealed ulcerative lesions of terminal ileum. Microscopic examination showed a severe ulcerating ileitis, without vasculitis granulomas. Search for infectious causes remained negative and cANCA were again positive (titre 500). A week later, bilateral pulmonary nodules with alveolar haemorrhage emerged.
Three pulses of i.v. methylprednisone together with 1 g i.v. of cyclophosphamide were administered with immediate efficacy on abdominal symptoms. One week later, colonoscopy was normal. The patient was then treated with prednisolone 1 mg/kg/day.
Comment.
Involvement of gastrointestinal tract has been described in 1024% of WG patients [2,3] (Table 1). Among those cases, intestinal manifestations were localized in any part of gastrointestinal tract. Acute abdominal pain, profuse diarrhoea with blood and mucus, peritonitis, and gut perforation appear to be the most common causes of immediate abdominal emergency. Colitis may be the initial presentation of WG [4]. It usually appears within the first 2 years of the disease [5]. The failure to demonstrate microscopic intestinal vasculitis in our patient could be due to the relatively small biopsy sample from the ileum. However, three of 20 reported cases of autopsied patients with gastrointestinal symptoms of WG disease showed no signs of ileal vasculitis in spite of deep enough sampling. To our knowledge, clinically prominent gastrointestinal symptoms have never been reported as a marker of WG disease relapse. Intestinal manifestations should alert the clinician as to a possible recurrence of WG disease.
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