Mayuko Fujisaki, Tomoaki Higuchi, Naoko Konda, Shinya Hirahara, Kotaro Watanabe, Risa Yamada, Ryo Motoyama, Rei Yamaguchi, Yasuhiro Katsumata, Yasushi Kawaguchi, Masayoshi Harigai
Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare type of idiopathic interstitial pneumonia, which is characterised by pleural fibrosis and subjacent parenchymal fibroelastosis of the upper lobes. Herein, we present a case of microscopic polyangiitis (MPA) following PPFE. The patient had abnormal shadows on chest radiographs 15 years before the onset of MPA, and the patient was diagnosed with PPFE. Four years after the PPFE diagnosis, the patient was diagnosed with MPA based on persistent fever, purpura, mononeuritis multiplex, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, and pathological findings of peritubular capillaritis on kidney biopsy...
June 12, 2023: Modern rheumatology case reports