A subset of patients with hypersensitivity pneumonitis (HP) develop lung fibrosis that is clinically similar to idiopathic pulmonary fibrosis (IPF). To address the aetiological determinants of fibrotic HP, we investigated whether the common IPF genetic risk variants were also relevant in study subjects with fibrotic HP. Our findings indicate that common genetic variants in TERC, DSP, MUC5B and IVD were significantly associated with fibrotic HP. These findings provide support for a shared etiology and pathogenesis between fibrotic HP and IPF.
- hypersensitivity pneumonitis
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Contributors IVY and DAS designed the study and provided quality control at each step of the project. HF, ADW, PLM, ERFP and PJW provided data and samples. HF performed the sample preparation. JSL, IVY and DAS supervised and coordinated the clinical and lab work assessment. HF, ALP and JC performed the data cleanings and analysis. HF and DAS wrote the original draft of the manuscript. All authors contributed to manuscript review, editing, and final approval for submission.
Funding This research was supported by grants from the NHLBI (R01-HL097163, R01-HL148437, P01- HL092870, UH3-HL123442 and UG3-HL151865) and DoD (W81XWH-17-1-0597).
Competing interests DAS reports grants from NIH-NHLBI, DOD, VAMC, and Eleven P15 during the conduct of the study; Travel support, patents, advisory board membership, ATS committee membership. Business collaboration with Eleven P15 and Vertex Pharmaceuticals. PJW reports grant from Boehringer Ingelheim during this study. ERFP reports grants from NIH-NHLBI, Colorado office of economic development and international trade research, Boehringer Ingelheim and Genentech during the study. Payment of honoraria for lectures from Boehringer Ingelheim speaker bureau. JSL reports a grant from NIH-NHLBI. ALP reports the consulting fee from ElevenP15. PLM is supported by an Action for Pulmonary Fibrosis Mike Bray fellowship and reports a grant from Boehringer Ingelheim, AstraZeneca and Roche. IVY, ADW, JC and HF declare no conflicts of interest associated with this manuscript.
Provenance and peer review Not commissioned; externally peer reviewed.
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