Abstract: [Abstract] Objective To explore the clicicopathologic features of granulomatosis with polyangiitis(GPA). Methods The clinical, laboratory analysis and radiologic features of one GPA case were analyzed retrospectively and the related literatures were reviewed. Results Computed tomography of the chest showed bilateral pulmonary multinodular high density shape with obscure margin, inhomogeneous density .Spicula and lobulated can be seen in surroundings, with part adhering to the pleural. Sinuses CT scanned left paranasal sinusitis. The laboratory analysis showed a positive test of anti-antineutrophil cytoplasmic antibody .The biopsy sample were got from nasal cavity operation and lung puncture. The pathological morphology presented with necrotizing granulomatous, local changes in vasculitis. Conclusion GPA is a kind of necrotizing granulomatous vasculitis, which involves respiratory, lung and kidney with low incidence and poor prognosis; The clinicians can choose biopsy in the case of laboratory and radiologic failed definitive diagnosis, for the timely diagnosis is the key of treatment.

Key words: Granulomatosis with polyangiitis, Granulomas, Vasculitis