Granulomatous gastritis in Wegener's disease: differentiation from Crohn's disease supported by a positive test for antineutrophil antibodies.

BACKGROUND: This report concerns the gastric manifestation of Wegener's granulomatosis in a 44 year old white female patient who initially presented with abdominal pain, vomiting, and iridocyclitis. FINDINGS: The clinical findings and the histopathological proof of granulomatous gastritis in the absence of necrotising vasculitis were initially considered to be indicative of a diagnosis of Crohn's disease showing isolated gastric involvement. A five month course of steroids resulted in temporary relief; thereafter the patient developed severe rhinitis with mucosal ulcerations. At this point biopsy of nasal mucosa disclosed the classic histopathological signs of Wegener's granulomatosis. A positive test for antineutrophil cytoplasmic antibodies (ANCAs) with a cytoplasmic pattern (c-ANCA) and antigenic specificity for proteinase 3 (PR-3) were found. The patient is in complete remission one year after diagnosis and treatment with steroids and cyclophosphamide. CONCLUSIONS: Wegener's granulomatosis can also involve the gastrointestinal tract. Granulomatous inflammation of the stomach, although a rare finding and non-specific, should include Wegener's disease in the differential diagnosis. The histological proof of necrotising vasculitis is dependent on the depth of the biopsy and therefore can be easily missed. Differential diagnosis can be clarified by ANCA testing.

[1]  R. Pounder,et al.  Crohn's disease: pathogenesis and persistent measles virus infection. , 1995, Gastroenterology.

[2]  F. Seibold,et al.  Neutrophil autoantibodies: a genetic marker in primary sclerosing cholangitis and ulcerative colitis. , 1994, Gastroenterology.

[3]  A. D'apice,et al.  Wegener's granulomatosis: clinical features and prognosis in 37 patients. , 1993, Australian and New Zealand journal of medicine.

[4]  F. Seibold,et al.  Clinical significance of antibodies against neutrophils in patients with inflammatory bowel disease and primary sclerosing cholangitis. , 1992, Gut.

[5]  Guy Hoffman,et al.  Wegener Granulomatosis: An Analysis of 158 Patients , 1992, Annals of Internal Medicine.

[6]  J. Schölmerich,et al.  [A new type of ANCA in sera of patients with ulcerative colitis: effects of therapy and disease severity on serum titer]. , 1992, Immunität und Infektion.

[7]  J. Pinkney,et al.  Gastrointestinal involvement in Wegener's granulomatosis. , 1991, Gastrointestinal endoscopy.

[8]  A. Dhillon,et al.  Granulomatous vasculitis in Crohn's disease. , 1991, Gastroenterology.

[9]  R. Tupler,et al.  Wegener granulomatosis of the colon: CT and histologic correlation. , 1991, Journal of computer assisted tomography.

[10]  R. Pounder,et al.  PATHOGENESIS OF CROHN'S DISEASE: MULTIFOCAL GASTROINTESTINAL INFARCTION , 1989, The Lancet.

[11]  C. Hack,et al.  Wegener's granulomatosis autoantibodies identify a novel diisopropylfluorophosphate-binding protein in the lysosomes of normal human neutrophils. , 1989, The Journal of clinical investigation.

[12]  N. Kurata,et al.  Small intestinal perforation in Wegener's granulomatosis. , 1989, The Journal of rheumatology.

[13]  I. Mackay,et al.  Intestinal perforation in Wegener's granulomatosis. , 1986, Gut.

[14]  C. Brown,et al.  Gastrointestinal haemorrhage complicating Wegener's granulomatosis. , 1985, British medical journal.

[15]  G. Littlejohn,et al.  Wegener's granulomatosis: clinical features and outcome in seventeen patients. , 1985, Australian and New Zealand journal of medicine.

[16]  T. H. The,et al.  AUTOANTIBODIES AGAINST NEUTROPHILS AND MONOCYTES: TOOL FOR DIAGNOSIS AND MARKER OF DISEASE ACTIVITY IN WEGENER'S GRANULOMATOSIS , 1985, The Lancet.

[17]  C. Pusey,et al.  Severe intestinal involvement in Wegener's granulomatosis. , 1984, Gut.

[18]  A. J. Mcadams,et al.  An unusual presentation of Wegener's granulomatosis mimicking inflammatory bowel disease. , 1984, Gastroenterology.

[19]  A. Rees,et al.  Wegener's granulomatosis: observations on 18 patients with severe renal disease. , 1983, The Quarterly journal of medicine.

[20]  J. Esdaile,et al.  Wegener's granulomatosis. Clinical features and outcome in 13 patients. , 1983, Archives of internal medicine.

[21]  B. Haynes,et al.  Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. , 1983, Annals of internal medicine.

[22]  W. Mcnabb,et al.  Small intestinal perforation in Wegener's granulomatosis , 1982, Postgraduate medical journal.

[23]  V. A. Lisovskii,et al.  [Wegener's granulomatosis]. , 1972, Klinicheskaia meditsina.

[24]  C. Carrington,et al.  Limited forms of angiitis and granulomatosis of Wegener's type. , 1966, The American journal of medicine.