2019•Unpublished venueRequires access
Idiopathic Pulmonary Fibrosis based on disease behavior: Workshop summary of a Japanese multicenter study
Yoshikazu Inoue, Masanori Akira, Takahiko Kasai, Toru Arai, Kazunobu Tachibana, Chikatoshi Sugimoto, Naoko Takeuchi, Yuki Matsui, Yoshinori Kawabata, Takeshi Johkoh, Fumikazu Sakai, Reiko Egashira, Hiromitsu Sumikawa, Shigeki Shimizu, Tomonori Tanaka, Tamiko Takemura, Shinyu Izumi, Keita Sakamoto, Shuichiro Matsumoto, Kazuhito Hatanaka, Goshi Matama, Tomohisa Baba, Ryobu Mori, Hiromi Tomioka, Keisuke Tomii, Naoki Hamada, Nobuhiko Nagata, Yasuhiro Kondoh, Kensuke Kataoka, Tomoo Kishaba, Yukihiro Nakamura, Akira Yamagata, Ayaka Tanaka, Venerino Poletti, Masanori Kitaichi
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Abstract
Background: Although the Fleischner Society’s white paper and the ATS/ERS/JRS/ALAT guideline for Idiopathic Pulmonary Fibrosis (IPF), 10-20% of Idiopathic Interstitial Pneumonias (IIPs) were diagnosed as unclassifiable IIPs and can be managed as IPF based on disease behaviour. Subject and Methods: To characterize such patients we collected clinical data, HRCT, and pathological slides of 25 cases with the final diagnosis of IPF {initial local diagnosis of non IPF and final IPF by disease behaviour {G1; n=17, 16 unclassifiable IIPs, 1 chronic hypersensitivity pneumonitis (CHP)} and initially definite IPF (G2; n=8)} from 10 centers. We reviewed in the IPF workshop according to the Fleischner Society criteria (2017) in August, 2018. Results: 41% (7/17) of initial non IPF were IPF (MDD) and 25% (2/8) of initial IPF were non IPF (CHP, unclassifiable). 25% (3/12) of Probable UIP (HRCT), and 67% (1/3) of Indeterminate for UIP were non IPF after MDD. Kappa statistic between the local diagnosis and MDD was 0.29. There was not significant difference of gender, age, smoking, dust exposure, %FVC, serum KL-6, SP-D, and honeycombing between G1 and G2. 100% of G1 and 88% of G2 progressed and developed more honeycombing in the last HRCT. 75% G1and 25% G2 were treated with Pirfenidone, and 53% G1 and 88% G2 were treated with Nintedanib. 63% G1 and 29% G2 developed acute exacerbation. 40% of G1 and 41% of G2 were deceased, 50% of G1 and 29% of G2 were alive with worsened disease. Discussion and Conclusion: Unclassifiable IIPs should be carefully managed by disease behaviour. Surgical lung biopsy and MDD should be conditionally required for patients with probable UIP (HRCT).