Satoshi Nagaoka, Yu Hara, Hiroaki Fujii, Kota Murohashi, Ryo Nagasawa, Yoichi Tagami, Yusuke Saigusa, Tatsuji Enomoto, Yutaka Matsumoto, Makoto Masuda, Nobuyuki Horita, Masaki Yamamoto, Nobuaki Kobayashi, Takashi Ogura, Takeshi Kaneko, Makoto Kudo
In patients with IPF treated with AFT, the radiological UIP pattern on HRCT was not independently associated with long-term prognosis. Multidimensional risk assessment incorporating physiological impairment and disease behavior may provide more accurate prognostic stratification in treated UIP populations.
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease with a poor prognosis. Honeycombing on high-resolution computed tomography (HRCT) is a defining feature of the usual interstitial pneumonia (UIP) pattern and has traditionally been considered a marker of advanced disease. However, in the era of antifibrotic treatment (AFT), the prognostic significance of the UIP pattern in IPF remains uncertain. This study aimed to evaluate the association between HRCT UIP pattern and long-term prognosis in patients with IPF receiving AFT.
METHODS: This retrospective multicenter study investigated consecutive patients with IPF who initiated AFT between 2009 and 2019 at seven institutions in Japan. HRCT patterns were classified as UIP or non-UIP by multidisciplinary assessment. The primary outcome was IPF-related events, defined as IPF-related death or first acute exacerbation (AE) after initiating AFT. Event-free survival was analyzed using Kaplan-Meier methods with stratification by gender-age-physiology (GAP) stage. Propensity score matching (PSM) was performed to adjust for age, sex, GAP score, and antifibrotic agent.
RESULTS: Of the 139 patients included, 99 had a UIP pattern, and 40 had a non-UIP pattern. In the overall cohort, event-free survival did not differ significantly. Stratified analyses showed no significant survival difference between UIP and non-UIP patterns among GAP Stage I patients (median survival time [MST]: 2050 vs. 1104 days; p = 0.90). Similarly, among patients with GAP Stages II-III, MST did not differ significantly. After PSM, 34 matched pairs were analyzed, and no significant differences in event-free survival were observed between UIP and non-UIP patterns.
CONCLUSIONS: In patients with IPF treated with AFT, the radiological UIP pattern on HRCT was not independently associated with long-term prognosis. Multidimensional risk assessment incorporating physiological impairment and disease behavior may provide more accurate prognostic stratification in treated UIP populations.