P0633 AN ADOLESCENT CASE OF WEGENER???S GRANULOMATOSIS MIMICKING INFLAMMATORY BOWEL DISEASE

Abstract

Introduction: WG is characterized by granulomatous vasculitis of the upper and lower respiratory tract together with glomerulonephritis. About 10% of the WG patients have some gastrointestinal symptoms, but only a few cases of WG have been reported which associated with IBD-like disorder. Methods: Case report Results: A twelve-year-old girl had high fever and arthralgia of both sides of knees and ankles. About one month later, severe abdominal pain and bloody diarrhea started. When she was admitted to our hospital, she had about 20 times of bloody diarrhea daily and had lost her body weight of 8 kg. She had oral and pharyngeal aphtha, anal fistula and scleritis. Blood examination showed that she had severe inflammation. Anti nucleic antibody and rheumatoid arthritis test were positive. Endoscopic evaluation disclosed that linear ulcer of ileum and sigmoid colon, and multiple round ulceration of total colon. Histological analysis showed that severe inflammation of lamina propria with neutrophil and lymphocyte infiltration and crypt distortion. Epithelioid granuloma and vasculitis were not detected. These findings suggested that she had inflammatory bowel disease (IBD). Intravenous hyperalimentation and elementary diet were undertaken for the treatment of malnutrition, and four courses of methylpredonisolone (mPSL) pulse therapy followed by oral predonisolone (PSL) 30mg, mesalazine 750mg and azathioprine 75mg were given. Her clinical and laboratory findings were improved smoothly in four months. Colonoscopy and histological analysis showed almost normal colonic mucosa. After that her laboratory data of inflammation were sometimes extremely elevated, but gastrointestinal symptoms never appeared. One year later it was recognized that the clinical course seemed to be atypical compared to that of IBD and that she had been suffering from chronic paranasal sinusitis. Endoscopic examination and biopsy of nasal cavity revealed that necrotizing granulomatous lesion with typical histological findings of Wegener’s granulomatosis (WG). Extremely high levels of peripheral anti-neutrophil-cytoplasmic-antibodies were also confirmed. Urinary examination was normal. Treatment for WG was introduced with one course of mPSL pulse therapy followed with oral cyclophosphamide(CPA) 50mg and PSL 60mg. Clinical and laboratory findings were improved smoothly, and now her remission had been kept over 15 weeks with the medication of CPA 25mg and PSL 35mg. Conclusion: Diffuse small vessel vasculitis in gastrointestinal tract may appear as systemic involvement of WG.