To the Editor:
Granulomatosis with polyangiitis (GPA; formerly Wegener’s granulomatosis) is a small to medium-size vessel vasculitis involving various organs, with the classic triad of upper airway, lung, and kidney involvement. GPA can be accompanied by chronic hypertrophic pachymeningitis (CHP), characterized by inflammatory thickening of the cerebral or spinal dura mater1. Although the pathology of CHP does not depend on the contiguous spread of granulomatous lesions from the nasal or paranasal sinuses, GPA with CHP has been reported to present mostly with limited organ involvement2,3. Specifically, antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive GPA with CHP has been reported to lack renal involvement1,2,4. We describe a case of MPO-ANCA-positive GPA, which presented with CHP-related central nervous system (CNS) symptoms and with renal abnormalities, with a discussion of the incidence of renal involvement in MPO-ANCA-positive GPA accompanied by CHP.
A previously healthy 48-year-old Japanese man presented to the otolaryngology department with a 3-month history of fever, bilateral temporal headaches, left facial nerve paralysis, dysphagia, and hoarseness, which occurred sequentially. …
Address correspondence to Dr. H. Kohsaka, Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima Bunkyo-ku, Tokyo 113-8519, Japan. E-mail: kohsaka.rheu{at}tmd.ac.jp