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Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society 2009-Nov

[A case of scleritis as the initial clinical manifestation of limited Wegener's granulomatosis].

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Naoya Hijikata
Noboru Takayanagi
Koichiro Yoneda
Yutaka Sugita
Yoshinori Kawabata

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A 63-year-old man who was transferred to our hospital had noticed ocular hyperemia, and 2 months later headache, dry cough, nose bleeds and fever. Chest X-ray film showed bilateral infiltrating shadows. He was given a diagnosis of pneumonia at the previous hospital, where antibiotics were administrated with no effect. High-resolution chest computed tomography (HRCT) on admission revealed consolidations distributed predominantly in the bilateral lower lobes and multiple nodules. Consolidations were also mainly distributed around bronchovascular bundles and at subpleural areas. We suspected Wegener's granulomatosis because of his eye lesion and HRCT findings. Ophthalmologic examination after admission revealed bilateral scleritis. The proteinase 3-antineutrophil cytoplasmic antibody level was 51.4 IU/ml. Thoracoscopic lung biopsy (left S8) showed eccentric granulomatous necrotizing vasculitis, which was consistent with Wegener's granulomatosis. We finally diagnosed this case as limited type Wegener's granulomatosis without renal involvement. We administrated both 60 mg/day prednisolone and 100mg/day cyclophosphamide for initial treatment. After this treatment, he remarkably improved. Scleritis was the initial clinical presentation in this case of Wegener's granulomatosis.

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