Kijlertsuphasri, Supiya

Background Antifibrotic (AF) therapy has been shown to potentially reduce the risk of lung cancer (LC) in patients with idiopathic pulmonary fibrosis (IPF). Our study further assesses the impact of AF use and LC incidence in a real-world cohort with patient-level data. Methods A retrospective multisite cohort study involving patients with IPF followed at Mayo Clinic between 2005 and 2022 was conducted. We identified individuals with new diagnoses of LC in the pre-AF and post-AF eras and defined AF use as continuous treatment for 6 months or more before LC diagnosis. Given the inclusion of LC cases prior to the wide availability of AF therapy and potential differences in patients exposed and unexposed to treatment, propensity score analysis with inverse probability of treatment weighting (IPTW) was used to balance comparator groups. Fine and Gray modelling was used to explore risk factors for developing LC, reported as parameter subdistribution HRs (SHR). Results A total of 3313 patients with IPF were included (1161 treated and 2152 non-treated). LC incidence rates were lower for treated patients in the post-AF era (0.34 vs 1.25 per 100 person-years, p<0.001). After IPTW, 2148 treated were compared with 2167 non-treated individuals. AF treatment was independently associated with reduced LC risk (SHR 0.36 (0.16–0.82), p=0.02), while smoking history and higher forced vital capacity were associated with increased risk. Conclusion AF use appears to be associated with a reduced incidence rate and risk of LC in patients with IPF.

Abstract Background: Birt-Hogg-Dube (BHD) syndrome is a rare genetic condition associated with the development of renal tumors. This study aims to determine typical age ranges for detecting renal abnormalities, risk factors for tumor development, and long-term outcomes based on current surveillance strategies. Methods: A single-center multi-site retrospective cohort study was performed on all patients with BHD diagnosed from 2000 to 2023. Baseline demographics, pulmonary function, laboratory, radiologic, and histopathologic findings were collected. Logistic regression was used to assess predictor variables for the development of renal tumors with survival analysis evaluated from the date of BHD diagnosis to date of death or last known follow-up. Results: The study included 149 patients with BHD, 39 (26%) with diagnosed renal tumors, of which 28 had histopathologic confirmation. Mean age at renal tumor detection was 53.61 years. Older age and male sex were predictive of renal tumor development ((odds ratio 1.05; 95% CI, 1.01-1.08, P = 0.002) and (odds ratio 2.59; 95% CI, 1.17-5.73, P=0.02), respectively). Time to all-cause mortality appeared shorter in those with renal tumors (Log-rank P = 0.02), though no deaths were from cancer or cancer-related complications. Conclusions: Current screening protocols for renal tumors in BHD suggest the most common presenting age range for presentation is late 40s to early 50s, with older age and male sex as risk factors for tumor development.