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- W4307714802 abstract "<b>Background:</b> 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. <b>Methods:</b> 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. <b>Results:</b> 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. <b>Conclusion:</b> 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. <i>Trial registration</i> retrospectively registered" @default.
- W4307714802 created "2022-11-05" @default.
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- W4307714802 date "2022-01-01" @default.
- W4307714802 modified "2023-09-26" @default.
- W4307714802 title "Fortgeschrittene pulmonale Sarkoidose: Auf Wirksamkeit und Zeitpunkt der antifibrotischen Therapie bei Patienten mit einem PF-ILD-Phänotyp konzentrieren" @default.
- W4307714802 doi "https://doi.org/10.1159/000527477" @default.
- W4307714802 hasPublicationYear "2022" @default.
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