DOI: 10.1097/01.MPG.0000155368.46841.21

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PMID: 15735495

Issn Print: 0277-2116

Publication Date: 2005/03/01


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An Unusual Intestinal Presentation of C-ANCA/PR-3 Positive Vasculitis in a Child

Daniel E DiMeo; Polly J Ferguson; Warren P Bishop

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Wegener's granulomatosis (WG) is an autoimmune disorder characterized by a necrotizing vasculitis of small to medium size vessels. The disease is rare in childhood. Affected individuals typically present with respiratory and renal involvement (1,2). Common presenting features include fever, malaise, sinusitis, epistaxis, arthritis, cough, hemoptysis and glomerulonephritis. Common cutaneous findings include ulcers and palpable purpura. Subglottic stenosis and nasal deformity are often seen (1). A positive anti-neutrophil cytoplasmic antibody (ANCA) with a cytoplasmic immunofluorescent staining pattern (c-ANCA) with specificity to proteinase 3 (PR-3) antigen is present in nearly all patients with WG and in a minority of patients with a similar vasculitic disorder, microscopic polyangiitis (MPA). The presence of necrotizing granulomas in affected tissues is diagnostic for WG. Angiography revealing evidence of vasculitis is also quite helpful (3,4).
Gastrointestinal manifestations are rare, especially at initial presentation and in isolation from other organ involvement. The incidence of gastrointestinal involvement ranges from 0% to 5% (5), with the most common symptoms including profuse diarrhea (6), abdominal pain (7) and bloody diarrhea (8). Many of the reported gastrointestinal symptoms have occurred in adults who have an established diagnosis of WG. These individuals typically have gastrointestinal complications secondary to intestinal wall ischemia, infarction with potential perforation, mucosal ulceration with potential gastrointestinal hemorrhage or aneurismal rupture (9-11). Gastric bleeding as a possible complication in adults with WG has been reported (11). Significant gastrointestinal involvement as a presenting symptom in a young child with Wegener's granulomatsosis is extremely rare. In 1984 Sokol et al. reported a 16-year-old female with WG whose initial presentation mimicked inflammatory bowel disease (12). We report an unusual intestinal presentation of ANCA-positive small vessel vasculitis in a 6-year-old girl.
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