Characterization of the PF-ILD phenotype in patients with advanced pulmonary sarcoidosis.

BACKGROUND: Advanced pulmonary sarcoidosis causes significant morbidity and can lead to death. Large trials demonstrated efficacy of antifibrotics in patients with progressive fibrosing interstitial lung diseases (PF-ILD), including a few with sarcoidosis. To date, little is known about this progressive fibrosing phenotype in sarcoidosis. Diffusion capacity of carbon monoxide (DLCO) may be a useful functional marker to screen for advanced pulmonary sarcoidosis. In this study, we describe a cohort with advanced pulmonary sarcoidosis and we gain insights in the progressive fibrosing phenotype in sarcoidosis. METHODS: Patients with sarcoidosis and a DLCO < 50% predicted were included in this retrospective cohort study. First measurement of DLCO < 50% predicted was the baseline. Lung function data, HRCT, pulmonary hypertension (PH) and mortality were collected. Patients with > 10% fibrosis on HRCT meeting the criteria for ILD-progression within 24 months were labelled as PF-ILD. With Cox-regression analysis predictors of mortality were established. RESULTS: 106 patients with a DLCO < 50% predicted were included. Evolution of forced vital capacity (FVC) varied widely between patients from - 34% to + 45% after 2 years follow-up, whereas change in DLCO varied between - 11% and + 26%. Fourteen patients (15%) met the PF-ILD criteria, of whom 6 (43%) died within 10 years versus 10 (13%) in the non PF-ILD group (p = 0.006). PH was present 12 (11%), 56 (53%) demonstrated > 10% fibrosis on HRCT. Independent predictors of mortality and lung transplantation in the whole cohort are PH, PF-ILD and UIP-like pattern. CONCLUSION: In conclusion, within this group with advanced pulmonary sarcoidosis disease course varied widely from great functional improvement to death. PF-ILD patients had higher mortality rate than the mortality in the overall pulmonary sarcoidosis group. Future research should focus on the addition of antifibrotics in these patients. Trial registration retrospectively registered.

Parents' perceptions of the services provided to children with cerebral palsy in the transition from preschool rehabilitation to school-based services.

AIM: To describe the course of parents' perceptions of the family centredness of rehabilitation services provided to their children with cerebral palsy (CP) before and after the transition from preschool to school-based services. BACKGROUND: Parents of 59 children with CP aged 2.5 to 4.5 years filled in the 56-item Measure of Processes of Care (MPOC-56) on three occasions pre (2) and post (1) transition to school-based services. Friedman tests were used to describe changes in parents' perceptions over time. Mann-Whitney U tests were used to describe differences in course of parents' perceptions between regular school and special school or day care. RESULTS: Parents' perceptions of preschool services were stable between the ages of 2.5 and 3.5 years, with a decline after transition on four of the five domains of the MPOC (P < 0.05). The domain providing general information was scored lowest (median at baseline 3.56, IQR 2.39) compared with the four other MPOC domains, but remained stable over time. No differences in course of parental perceptions were found for school type. CONCLUSION: The transition from preschool to school-based services for children with CP is associated with a decrease in parents' perception of family centredness independent of the type of school. The transition in services has a negative impact on perceived family-centred practices.