[A case of eosinophilic granulomatosis with polyangiitis with extra-vascular granuloma and eosinophilic vasculitis diagnosed by transbronchial lung biopsy].

ABSTRACT

A 62-year-old man was suffering from bronchial asthma and referred to our institution with dry cough and dyspnea on exertion in November, 2010. He was diagnosed with eosinophilic granulomatosis with polyangiitis (EPGA, formerly Churg-Strauss syndrome) by chest radiographic findings, blood eosinophilia, mononeuritis multiplex and cardiomyopathy. Steroid therapy was started and he was rapidly improved. Steroid therapy had been tapered off by May, 2012. After 2 months, however, progressive dyspnea, neural symptoms, deafness, re-elevation of blood eosinophils and bilateral multifocal infiltrations appeared. He was re-admitted to our institution. Transbronchial lung biopsy (TBLB) specimens revealed extra-vascular granuloma, eosinophilic vasculitis and eosinophilic pneumonia and we diagnosed him with the reccurence of EGPA. He was improved by steroid pulse therapy, then tapered. This case was the antineutrophil cytoplasmic autoantibodies negative EGPA. The case of EGPA with granuloma and vasculitis diagnosed by TBLB was rare.