[A case of Wegener's granulomatosis with episcleritis, mediastinal lesions, and pleural effusion and without serum anti-neutrophil cytoplasmic antibodies].

We report a 63-year-old male case of Wegener's granulomatosis. His complaints were cough and fever, and chest X-ray showed subpleural infiltrative opacities in the bilateral lungs and small nodular lesions in the middle portion of the right lung. He had been treated as bacterial pneumonia at another hospital without any improvement and was referred to this hospital. Chest CT scan disclosed several small nodular lesions in the subpleural region of the right lung, consolidation in the posterior mediastinum, and the left pleural effusion. Transbronchial lung biopsy revealed inflammatory granulation with predominantly lymphocytes and micro-necrosis in the peribronchiolar region. Although these findings suggested Wegener's granulomatosis, open lung biopsy was performed because both antineutrophil cytoplasmic antibodies (ANCA) and antibodies to proteinase-3 were negative and ear, nose, and throat abnormalities were not found. Pathologic findings consisted mainly of granulomatous inflammation with lymphocytes, histiocytes, and multinucleated giant cells, and granulomatous vasculitis with micro-necrosis. Furthermore, the patient was diagnosed as having bilateral episcleritis by an ophthalmologist. No renal involvement was found. Standard treatment with cyclophosphamide and glucocorticoid resulted in marked improvement within a couple of weeks, and the patient was in complete remission eight months after initiation of therapy. This case is of interest in that the distribution of lesions was not typical and ANCA was negative.