Pulmonary fibrosis pathophysiology is increasingly recognized to be influenced by the biology of telomeres, a field that has now grown in prominence with the widespread adoption of affordable next-generation sequencing over the past decade, leading to an improved understanding of telomere-related mechanisms underlying pulmonary fibrosis. Despite these advancements, notable gaps in knowledge persist. Firstly, individuals with telomere biology disorders or shortening face a heightened risk of developing conditions such as fibrotic interstitial lung diseases, but the genetic and molecular mechanisms catalyzing this process are yet to be fully elucidated. Secondly, the field lacks comprehensive natural history studies focusing on telomere-related mechanisms underlying the progression of pulmonary fibrosis to death or lung transplantation across diverse racial groups. Thirdly, the current ability to model telomere-related pulmonary fibrosis is constrained due to the absence of high-fidelity models. Lastly, and perhaps most significantly, there is an evident paucity in our understanding of the implications of racial diversity in genetic and genomic research, especially as it impacts disease progression in pulmonary fibrosis. Numerous individuals displaying pulmonary fibrosis phenotypes post-genomic sequencing do not exhibit commonly identifiable genetic markers, suggesting the potential discovery of novel, racially diverse telomere-related risk loci for pulmonary fibrosis syndromes is on the horizon. In my doctoral research, I have addressed pivotal knowledge gaps by conducting an unprecedented large-scale natural history study, exploring the progression of pulmonary fibrosis across diverse racial groups, leading to either death or lung transplantation. I collaborated with several institutions to investigate telomere length's predictive capacity in racially diverse pulmonary fibrosis cohorts, uncovering stark racial disparities, especially among Black patients. I found that peripheral leukocyte telomere length consistently correlates with chronological age and serves as a predictive mortality biomarker in pulmonary fibrosis across all racial groups. Further, I delved into the nuances of telomere biology in peripheral blood mononuclear cell subsets and induced pluripotent stem cells (iPSCs) derived from multiracial cohorts of patients with pulmonary fibrosis. My research revealed the prognostic impact of telomere length in patients with fibrotic hypersensitivity pneumonitis receiving immunomodulatory therapy, marking a pioneering study in this domain. My findings identified significant mutations in eight genes—PDE4DIPP, ZNF683, SFRP5, MIR6077, RPSAP72, WASIR2, GAPDHP27, and CNTNAP3P2—as critical drivers of honeycomb fibrosis in multiethnic populations with pulmonary fibrosis. This study unveiled unique transcriptomic differences in key variants across Black and White patients. Through a comprehensive integration of whole genome sequencing and expression quantitative trait loci analyses, I have revealed striking differences between self-identified race and genetic ancestry and uncovered novel host defense and cell senescence gene variants contributing to disease risk across a spectrum of pulmonary fibrosis subtypes. In sum, my findings regarding telomere biology and genomic risk variants in pulmonary fibrosis have yielded new insights into disease progression mechanisms, enhanced theragnostic modeling, fostered international and institutional data sharing and collaboration, and generated patient-derived cellular models for future research into disease mechanisms and innovative therapeutic strategies specific to pulmonary fibrosis.