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( Sung Woo Moon ),( Song Yee Kim ),( Behalf Of Ild Study Group ),( Moo Suk Park ) 대한결핵 및 호흡기학회 2019 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.127 No.-
Background: The Korean Interstitial Lung Disease Study Group created two nationwide, multicentre consecutive registries on idiopathic pulmonary fibrosis (IPF). Comparing these registries, this study aimed to evaluate the long-term change in clinical features, diagnostic modalities, and prognostic factors of IPF in the real world. Methods: We enrolled patients from the first registry (2008 group, January 2002-September 2008) and the second registry (2018 group, January 2012-August 2018). Survival curves were estimated using the Kaplan-Meier method. To evaluate the risk factor for the mortality in each registry, Cox regression models were used. Results: The 2008 and the 2018 groups comprised 1839 and 1345 patients, respectively. The 2018 group was younger (P=0.025), had fewer symptoms (P<0.001), had less honeycombing (P<0.001), and was less frequently diagnosed with surgical biopsy (P<0.001). Further, in the 2018 group, steroid use and conservative care declined, while the N-acetylcysteine use increased. Pirfenidone and nintedanib were only used in the 2018 group. 2018 group showed better survival. There was no significant difference in survival between the 2008 group and the 2018 subgroup group who did not use anti-fibrotic agent (P=0.197). In the evaluation of the risk factor for mortality in each registry, significant factors were higher gender-age-physiologic (GAP) score, and history of cancer in the 2008 group, while they were higher GAP score, history of hepatitis, history of cancer, honeycombing on HRCT, and steroid use in the 2018 group. Pirfenidone use (P<0.001) was related with lower mortality in the 2018 group. Conclusions: Actual clinical practice in the diagnosis and treatment of IPF patients have changed in adherence with guidelines, novel medications, and social circumstances, and the survival of IPF patients have improved. Proper guidelines for the diagnosis and management of IPF based on accumulated data and observations are crucial and must be combined with newer treatment.
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