BackgroundThe development of connective tissue disease‐associated lung diseases (CTD‐LD) occurs in association with specific human leukocyte antigens (HLA). For CTD‐LD patients who require lung transplant, it is unknown whether utilization of donor organs expressing these same HLA impacts posttransplant outcomes.MethodsUsing the Scientific Registry of Transplant Recipients, we assessed whether CTD‐LD lung transplant recipients in the United States have worse bronchiolitis obliterans (BOS)‐free survival based on the degree of donor HLA matching. This included overall degree of donor‐recipient HLA matching, donor‐recipient matching at DR loci, and recipient matching with specific donor HLA antigens associated with the development of pulmonary disease in their condition.ResultsAmong 1413 patients with CTD‐ILD, highly HLA‐matched donor‐recipients did not have worse adjusted survival (hazard ratio [HR] = 0.93, 95% confidence interval [CI] = 0.58–1.51, p = 0.77). Recipients who were fully matched at HLA DR did not have worse survival (HR = 0.82, 95% CI = 0.56–1.19, p = 0.29). Finally, among individual CTD‐LD, including rheumatoid arthritis, systemic sclerosis, the idiopathic inflammatory myopathies, and systemic lupus erythematous, transplant with a donor expressing HLA antigens associated with lung manifestations in these conditions was not associated with worse BOS‐free survival.ConclusionsAmong transplant recipients with CTD‐LD, HLA donor‐recipient matching, including at the DR loci, does not result in worse BOS‐free survival. Based on these findings, there is no reason to treat these as unacceptable antigens when considering donor offers for CTD‐LD candidates.