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Interstitial Lung Disease

Disease course varies greatly in patients with advanced pulmonary sarcoidosis

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A widely varying disease course was seen in patients with advanced pulmonary sarcoidosis, with patients with progressive fibrosing interstitial lung diseases (PF-ILD) having a higher mortality rate than the overall pulmonary sarcoidosis rate, according to a study.

This retrospective cohort study included 106 with sarcoidosis and a diffusion capacity of carbon monoxide (DLCO) < 50% predicted. Baseline was considered the first measurement of DLCO < 50% predicted.

After 2 years of follow-up, there was a wide variation in the evolution of forced vital capacity (FVC) from – 34% to + 45%. DLCO, however, varied between – 11% and + 26%.

Out of the 106 patients, 14 met the criteria for PF-ILD. Of those 14, 43% died within 10 years compared with 13% in the non PF-ILD group. Pulmonary hypertension was noted in 11% and 53% demonstrated >10% fibrosis on high-resolution computed tomography.

For the whole cohort, independent predictors of mortality and lung transplantation were pulmonary hypertension, PF-ILD, and UIP-like pattern.

Reference
Schimmelpennink MC, Meek DB, Vorselaars ADM, et al. Characterization of the PF-ILD phenotype in patients with advanced pulmonary sarcoidosis. Respir Res. 2022;23(1):169. doi: 10.1186/s12931-022-02094-7. PMID: 35752806.

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