, Volume 7, Issue 3, pp 183-188

A case of nasal T cell lymphoma with lethal midline granuloma which is clinically indistinguishable from Wegener’s granulomatosis

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We describe a patient who developed fever, bilateral otitis media, destructive necrosis of the nasal cavity and multiple lung nodules. The patient fulfilled the American College of Rheumatology (ACR) classification criteria for Wegener’s granulomatosis (WG) and was also diagnosed as having WG by using the ACR classification tree. However, the diagnosis of T cell lymphoma was finally made by cervical lymph node biopsy, 2 years after disease onset. Rheumatologists should therefore aware of the pitfall of using diagnostic criteria and repetitive biopsy is strongly recommended for accurate diagnosis of WG.