[Granulomatosis with Polyangiitis: Diagnosis and Treatment Strategies]
1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.
Abstract:
Granulomatosis with polyangiitis (GPA) is designated as an intractable disease by the Ministry of Health, Labor and Welfare, Japan, and is classified as an antineutrophil cytoplasmic antibody (ANCA)-related vasculitis syndrome. It is associated with upper respiratory tract symptoms (E; ear and nose), pulmonary symptoms (L; lung), renal symptoms (K; kidney), and systemic vasculitis symptoms, and often involves the central/peripheral nervous system. Patients with GPA can be easily diagnosed as they often show positive serum C (Proteinase 3)-ANCA findings. Remission can be induced using multiple immunosuppressants in combination, but caution is required as relapse and infection is common in patients with GPA.
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