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Adult patients with CF show a limited exercise capacity with lower peak oxygen consumption and prolonged oxygen kinetics. Interestingly, decreased IC qualified as the only significant predictor of exercise capacity in our study.

Introduction Multiple sclerosis (MS) is a highly heterogeneous inflammatory disease of the central nervous system. Patient-reported outcomes (PROs) in a real-world clinical setting can provide detailed information about MS from the patient's perspective. PROs were used here to assess quality of life (QoL), treatment satisfaction, clinical efficacy, and safety outcomes in a Greek cohort of relapsing remitting MS (RRMS) patients treated with oral teriflunomide (14 mg/day). Methods AURELIO was a 2-year, prospective, observational study whose QoL primary endpoint was assessed with the Multiple Sclerosis Impact Scale (MSIS-29). Secondary endpoints included analyses of Patient Determined Disease Steps (PDDS), Treatment Satisfaction Questionnaire for Medication (TSQM), Expanded Disability Status Scale (EDSS), annualized relapse rate (ARR), adherence, and safety outcomes. Results AURELIO enrolled 282 patients (62.8% female; mean age 44.8 [SD ± 11] years; EDSS 2.0 [SD ± 1.6]; 44.6% treatment-naïve), with 212 patients (75%) remaining on treatment at study end. MSIS-29 total scores remained stable, while the MSIS-29 psychological scale showed significant improvement ( p = 0.0015) at 2 years vs. baseline. TSQM scores at 2 years showed significant improvements in effectiveness (+ 6.6, p = 0.0001), convenience (+ 1.9, p = 0.0256), and global satisfaction (+ 8.1, p = 0.0001) vs. baseline. Disease progression was stable as indicated by non-significant changes in PDDS and EDSS vs. baseline. The ARR was low at 0.065, with a slightly higher ARR in previously treated (0.070) vs. naïve patients (0.058). Adherence was high at > 90%. Overall, 91 patients (32.3%) in the study reported a total of 215 safety events (32 serious, of which 21 were classified as mild–moderate). No new safety signals were observed. Conclusions These data highlight the importance of PROs to facilitate personalized treatment strategies in MS. In line with other teriflunomide studies, AURELIO showed stable QoL, efficacy and safety outcomes, and good treatment satisfaction both in treatment-naïve and previously treated patients in this Greek cohort of patients with RRMS. Supplementary Information The online version contains supplementary material available at 10.1007/s40120-022-00384-2.

BACKGROUND:The goal of this study was to explore the relation between oxygen kinetics during constant work load submaximal cardiopulmonary exercise test (CPET) and disease severity in adult subjects with cystic fibrosis. METHODS: Fourteen adult subjects with cystic fibrosis (CF; 8 males, 22 ؎ 4 y old) and a mean Schwachman score of 73 ؎ 11 and 10 healthy individuals (5 males, 29 ؎ 4 y old) underwent pulmonary function tests at rest, maximal and constant work load submaximal CPET on a cycloergometer. Breath-by-breath analysis was used for measuring oxygen kinetic parameters and the time constant (tau), expressing phase 2 of submaximal CPET. RESULTS: Subjects with CF had a significantly prolonged tau compared with healthy subjects (42.3 ؎ 21.5 vs 29.3 ؎ 6.4, s, P < .05). The tau during phase 2 was inversely correlated with FEV 1 (% pred) (r ‫؍‬ ؊0.77, P ‫؍‬ .001), breathing reserve (r ‫؍‬ ؊0.74, P ‫؍‬ .003), V O2peak (r ‫؍‬ ؊0.53, P ‫؍‬ .049), V O2 /t slope (r ‫؍‬ ؊0.58, P ‫؍‬ .03), and Schwachman score (r ‫؍‬ ؊0.80, P ‫؍‬ .001). In a multivariate regression model including all the above variables, the Schwachman score (␤ ‫؍‬ ؊0.697, P ‫؍‬ .002) emerged as independent predictor of tau (R 2 ‫؍‬ 0.719, P ‫؍‬ .001). CONCLUSIONS: We conclude that adult subjects with CF present significant prolonged oxygen kinetics during constant work load submaximal exercise in relation to disease severity. Thus, submaximal exercise should be considered the preferable CPET choice in adult patients with severe CF.

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