American journal of respiratory and critical care medicine
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Am. J. Respir. Crit. Care Med. · Apr 2020
Follistatin-like 1 Attenuation Causes Spontaneous Smoke-Resistant Pulmonary Emphysema.
Rationale: The role of FSTL-1 (follistatin-like 1) in lung homeostasis is unknown. Objectives: We aimed to define the impact of FSTL-1 attenuation on lung structure and function and to identify FSTL-1-regulated transcriptional pathways in the lung. Further, we aimed to analyze the association of FSTL-1 SNPs with lung disease. ⋯ Conclusions: This work identifies a novel role for FSTL-1 protecting against emphysema development independent of smoke exposure. This FSTL-1-deficient emphysema implicates regulation of immune tolerance in lung macrophages through Nr4a1. Further study of the mechanisms involving FSTL-1 in lung homeostasis, immune regulation, and NF-κB signaling may provide additional insight into the pathophysiology of emphysema and inflammatory lung diseases.
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Am. J. Respir. Crit. Care Med. · Apr 2020
Income and Other Contributors to Poor Outcomes in US Sarcoidosis Patients.
Rationale: Socioeconomic factors are associated with worse disease severity at presentation in sarcoidosis, but the relative importance of socioeconomic variables on morbidity and disease burden has not been fully elucidated. Objectives: To determine the association between income and sarcoidosis outcomes after controlling for socioeconomic and disease-related factors. Methods: Using the Sarcoidosis Advanced Registry for Cures database, we analyzed data from 2,318 patients with sarcoidosis in the United States to determine the effect of income and other variables on outcomes. ⋯ Development of comorbidities after diagnosis of sarcoidosis occurred in 63% of patients and were strong independent predictors of poor outcomes. In random forest modeling, income was consistently a leading predictor of outcome. Conclusions: These results suggest the burden from sarcoidosis preferentially impacts the economically disadvantaged.