In two small retrospective studies, ERT has been reported to stabilize or even ameliorate the burden of pulmonary involvement in Fabry disease, mainly bronchial obstruction and/or sleep apnea [20,21]. An interventional study of six patients receiving either ERT or placebo showed an increased exercise tolerance in patients receiving ERT [22]. Lung function parameter in five patients (2 males) on ERT were followed longitudinally for an average of five years [17]. In three patients, FVC and FEV1 increased during the treatment period, in one patient slight decrease of FVC and FEV1 were registered and in one patient a slight decrease in FVC, with an increase in FEV1. Recently, Franzen et al. [23] reported the change in z-scores of FEV1 and FEV1/FVC over time in 53 patients. In the same study, they investigated whether factors related to age, sex, tobacco, phenotype, residual α-gal activity, age at ERT initiation, Mainz Severity Score Index (MSSI) and lyso-Gb3 levels in plasma may affect change in FEV1 and FEV1/FVC over time. Median spirometric follow-up time was 7.7 years. When considering z-scores, 27 patients (51%) developed airflow limitation over time. Factors including male sex, active tobacco or past history of smoking, cardiac involvement and increased patient age at ERT initiation were associated with faster FEV1 decline in the univariate analysis. These results suggest that earlier ERT initiation may help to stabilize FEV1 decline and preserve pulmonary function.