Radiological and Physiological Predictors of IPF Mortality

Abstract

Background and Objectives: Idiopathic pulmonary fibrosis (IPF) has a variable clinical course, which ranges from being asymptomatic to progressive respiratory failure. The purpose of this study was to evaluate the novel clinical parameters of IPF patients who receive an anti-fibrotic agent. Materials and Methods: From January 2011 to January 2021, we identified 39 IPF patients at Okinawa Chubu Hospital. Clinical information was obtained, such as laboratory data, pulmonary function test (PFT) results, and chest images, including of soft tissue thickness and the high-resolution computed tomography (HRCT) pattern at diagnosis. Results: The mean age was 72.9 ± 7.0 (53–85); 27 patients were men and 12 were women. The mean body mass index was 25.1 ± 3.9 (17.3–35). Twenty-four were active smokers and the median number of packs per year was 20. Regarding laboratory findings, mean white blood cell (WBC), lactate dehydrogenase (LDH), and Krebs Von den Lungen-6 (KL-6) values were 7816 ± 1859, 248 ± 47, and 1615 ± 1503, respectively. In PFT, the mean percent predicted FVC, percent predicted total lung capacity, percent predicted functional residual capacity (FRC), and percent predicted diffusion capacity of the lung for carbon monoxide (DLco) were 66.8 ± 14.9%, 71.8 ± 13.7%, 65 ± 39.6%, and 64.6 ± 27.9%, respectively. In chest radiological findings, soft tissue thickness at the right 9th rib was 26.4 ± 8.8 mm. Regarding chest HRCT patterns, 15 showed the definite usual interstitial pneumonia (UIP) pattern, 16 showed the probable UIP pattern, and eight showed the indeterminate for UIP pattern. In the treatment, 24 patients received pirfenidone and 15 patients took nintedanib. The mean observation period was 38.6 ± 30.6 months and 24 patients died. The median survival time was 32.4 months (0.9–142.5). Multivariate analysis adjusted for age showed that both soft tissue thickness [Hazard ratio (HR): 0.912, 95% confidence interval (CI): 0.859–0.979, p-value: 0.009] and percent FRC [HR: 0.980, 95% CI: 0.967–0.992, p-value: 0.002] were robust predictors of IPF mortality. Conclusions: In IPF patients treated with anti-fibrotic agents, both soft tissue thickness at the right 9th rib shown on the chest radiograph and %FRC can be novel predictors of IPF mortality.

Presence of focal usual interstitial pneumonia is a key prognostic factor in progressive pulmonary fibrosis

“…19,20 UIP-positive patients would be expected to display a downward trend in FVC, DLco and higher KL-6 values in the diseased population, but our cohort is made up of many different aetiologies, including subacute conditions, and thus the reverse trend in FVC and the lack of significance in DLco and KL-6 was not ultimately surprising. 21,22 The lack of significance in these variables highlights the many confounding factors present in cases of PPF.…”

Focal Usual Interstitial Pneumonia-like Fibrosis is a Core Prognostic Factor in Progressive Pulmonary Fibrosis

“…Significance was observed in the prevalence of focal UIP in smokers vs. non- smokers, which was predictable as the criteria for focal UIP positivity was lower than that for pathological UIP; we thus assumed greater stratification between diseased and non-diseased patients. Although a patient cohort comprised entirely of typical UIP positive patients would be expected to display a downward trend in FVC, DLco, and higher KL-6 values in the diseased population, our cohort is made up of many different etiologies, including subacute conditions, and thus the reverse trend in FVC and the lack of significance in DLco and KL-6 was not ultimately surprising [21,22]. The lack of significance in these variables highlights the many confounding factors present in cases of PPF.…”