Ionizing radiation is an established carcinogen, but its effects on non-malignant respiratory disease (NMRD) are less clear. Cohorts exposed to multiple risk factors including radiation and toxic dusts conflate these relationships, and there is a need for clarity in previous findings. This systematic review was conducted to survey the body of existing evidence for radiation effects on NMRD in global nuclear worker cohorts. A PubMed search was conducted for studies with terms relating to radiation or uranium and noncancer respiratory outcomes. Papers were limited to the most recent report within a single cohort published between January 2000 and December 2020. Publication quality was assessed based upon UNSCEAR 2017 criteria. In total, 31 papers were reviewed. Studies included 29 retrospective cohorts, one prospective cohort, and one longitudinal cohort primarily comprising White men from the U.S., Canada and Western Europe. Ten studies contained subpopulations of uranium miners or millers. Papers reported standardized mortality ratio (SMR) analyses, regression analyses, or both. Neither SMR nor regression analyses consistently showed a relationship between radiation exposure and NMRD. A meta-analysis of excess relative risks (ERRs) for NMRD did not present evidence for a dose-response (overall ERR/Sv: 0.07; 95% CI: –0.07, 0.21), and results for more specific outcomes were inconsistent. Significantly elevated SMRs for NMRD overall were observed in two studies among the subpopulation of uranium miners and millers (combined n = 4229; SMR 1.42–1.43), indicating this association may be limited to mining and milling populations and may not extend to other nuclear workers. A quality review showed limited capacity of 17 out of 31 studies conducted to provide evidence for a causal relationship between radiation and NMRD; the higher-quality studies showed no consistent relationship. All elevated NMRD SMRs were among mining and milling cohorts, indicating different exposure profiles between mining and non-mining cohorts; future pooled cohorts should adjust for mining exposures or address mining cohorts separately.