Articles: interstitial-lung-diseases.
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Am. J. Respir. Crit. Care Med. · Dec 2024
Development, Progression, and Mortality of Suspected Interstitial Lung Disease in COPDGene.
Rationale: Some with interstitial lung abnormalities (ILA) are suspected to have interstitial lung disease (ILD), a subgroup with adverse outcomes. Rates of development and progression of suspected ILD and their effect on mortality are unknown. Objectives: To determine rates of development, progression, and mortality in those with suspected ILD and assess effects of individual ILD and progression criteria. ⋯ Mortality rates were 31%, 50%, 45%, and 45%, respectively; those with only CT progression had worse mortality than those with ILA (HR, 2.6; P = 0.005). At 5-year follow-up, incident ILD occurred in 148/4,842 participants without prevalent ILD (5.5/1,000 person-years) and had worse mortality than ILA (HR, 2.4; P < 0.001). Conclusion: Rates of mortality and progression are high among those with suspected ILD in COPDGene; fibrosis and radiologic progression are important predictors of mortality.
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Am. J. Respir. Crit. Care Med. · Dec 2024
Progressive Early Interstitial Lung Abnormalities in Persons At-Risk for Familial Pulmonary Fibrosis: A Prospective Cohort Study.
Rationale: Relatives of patients with familial pulmonary fibrosis (FPF) are at increased risk to develop FPF. Interstitial lung abnormalities (ILAs) are a radiologic biomarker of subclinical disease, but the implications of very mild abnormalities remain unclear. Objectives: To quantify the progression risk among FPF relatives with abnormalities below the threshold for ILAs as described by the Fleischner Society and to describe the characteristics of participants with new or progressive ILAs during observation. ⋯ During a mean follow-up period of 6.2 ± 3.0 years, progression occurred among 31 of 211 (15%) of those with absence of ILAs at enrollment, 32 of 49 (65%) of those with mild ILAs, and 10 of 13 (77%) of those with moderate ILAs. Subjects with mild ILAs had 9.15 (95% confidence interval, 4.40-19.00; P < 0.0001) times and those with moderate ILAs had 17.14 (95% confidence interval, 4.42-66.49; P < 0.0001) times the odds of progression as subjects without ILAs. Conclusions: In persons at risk for FPF, minor interstitial abnormalities, including reticulation that is unilateral or involves <5% of a lung zone, frequently represent subclinical disease.
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The screening strategy for interstitial lung disease (ILD) in patients with rheumatoid arthritis is currently debated. Although high-resolution CT (HRCT) imaging is the gold standard for diagnosing ILD, its systematic use as a screening tool is not yet recommended. The role of lung ultrasound (LUS) in assessing ILD has been previously explored. ⋯ LUS is a promising tool for ILD screening in asymptomatic patients with rheumatoid arthritis, offering high sensitivity and negative predictive value. Its incorporation into routine clinical practice could optimize ILD screening strategies and enhance patient outcomes through early detection and intervention.
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Nintedanib reduces the decline of pulmonary function in patients with advancing lung fibrosis. ⋯ Nintedanib emerges as a promising therapeutic agent compatible with immunosuppressives for treating progressive lung fibrosis in patients with CTD-related ILD.