Claims-based Prevalence of Disease Progression among Patients with Non-IPF Fibrosing Interstitial Lung Disease in the US

Leukocyte telomere length (LTL), MUC5B rs35705950 and TOLLIP rs5743890 have been associated with idiopathic pulmonary fibrosis (IPF).In this observational cohort study, we assessed the associations between these genomic markers and outcomes of survival and rate of disease progression in patients with interstitial pneumonia with autoimmune features (IPAF, n=250) and connective tissue disease-associated interstitial lung disease (CTD-ILD, n=248). IPF (n=499) was used as a comparator.The LTL of IPAF and CTD-ILD patients (mean age-adjusted log-transformed T/S of −0.05±0.29 and −0.04±0.25, respectively) is longer than that of IPF patients (−0.17±0.32). For IPAF patients, LTL <10th percentile is associated with faster lung function decline compared to LTL ≥10th percentile (−6.43% per year versus −0.86% per year; p<0.0001) and worse transplant-free survival (hazard ratio 2.97, 95% CI 1.70–5.20; p=0.00014). The MUC5B rs35705950 minor allele frequency (MAF) is greater for IPAF patients (23.2, 95% CI 18.8–28.2; p<0.0001) than controls and is associated with worse transplant-free IPAF survival (hazard ratio 1.92, 95% CI 1.18–3.13; p=0.0091). Rheumatoid arthritis (RA)-associated ILD (RA-ILD) has a shorter LTL than non-RA CTD-ILD (−0.14±0.27 versus −0.01±0.23; p=0.00055) and higher MUC5B MAF (34.6, 95% CI 24.4–46.3 versus 14.1, 95% CI 9.8–20.0; p=0.00025). Neither LTL nor MUC5B are associated with transplant-free CTD-ILD survival.LTL and MUC5B MAF have different associations with lung function progression and survival for IPAF and CTD-ILD.

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Assessment of disease progression in systemic sclerosis-associated interstitial lung disease (SSc-ILD) patients using Functional Respiratory Imaging (FRI)

10.1183/13993003.congress-2019.pa4805 ◽

2019 ◽

Author(s):

Johan Clukers ◽

Kate Homer ◽

Maarten Lanclus ◽

Dennis Belmans ◽

Cedric Van Holsbeke ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Disease Progression

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AB1279 Evaluation of different composite radiological indexes for assessment of interstitial lung disease progression in systemic sclerosis:

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2012-eular.1275 ◽

2013 ◽

Vol 71 (Suppl 3) ◽

pp. 710.17-710

Author(s):

L.P. Ananyeva ◽

V. Lesnyak ◽

O. Ovsyannikova ◽

O. Koneva ◽

D. Goryachev

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Disease Progression

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Predictors of progression in systemic sclerosis patients with interstitial lung disease

European Respiratory Journal ◽

10.1183/13993003.02026-2019 ◽

2020 ◽

Vol 55 (5) ◽

pp. 1902026 ◽

Cited By ~ 11

Author(s):

Oliver Distler ◽

Shervin Assassi ◽

Vincent Cottin ◽

Maurizio Cutolo ◽

Sonye K. Danoff ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Disease Progression ◽

Topoisomerase I ◽

Diagnostic Tools ◽

Systemic Autoimmune Disease ◽

High Resolution Computed Tomography ◽

Time Period ◽

Organ Systems

Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5–9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.

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A cluster-based analysis evaluating the impact of comorbidities in fibrotic interstitial lung disease

Respiratory Research ◽

10.1186/s12931-020-01579-7 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

Alyson W. Wong ◽

Tae Yoon Lee ◽

Kerri A. Johannson ◽

Deborah Assayag ◽

Julie Morisset ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Lung Function ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Disease Progression ◽

Rapid Decline ◽

Agglomerative Clustering ◽

Lung Function Decline ◽

Obstructive Sleep ◽

The Impact

Abstract Background Comorbidities are frequent and have been associated with poor quality of life, increased hospitalizations, and mortality in patients with interstitial lung disease (ILD). However, it is unclear how comorbidities lead to these negative outcomes and whether they could influence ILD disease progression. The goal of this study was to identify clusters of patients based on similar comorbidity profiles and to determine whether these clusters were associated with rate of lung function decline and/or mortality. Methods Patients with a major fibrotic ILD (idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis, connective tissue disease-associated ILD, and unclassifiable ILD) from the CAnadian REgistry for Pulmonary Fibrosis (CARE-PF) were included. Hierarchical agglomerative clustering of comorbidities, age, sex, and smoking pack-years was conducted for each ILD subtype to identify combinations of these features that frequently occurred together in patients. The association between clusters and change in lung function over time was determined using linear mixed effects modeling, with adjustment for age, sex, and smoking pack-years. Kaplan Meier curves were used to assess differences in survival between the clusters. Results Discrete clusters were identified within each fibrotic ILD. In IPF, males with obstructive sleep apnea (OSA) had more rapid decline in FVC %-predicted (− 11.9% per year [95% CI − 15.3, − 8.5]) compared to females without any comorbidities (− 8.1% per year [95% CI − 13.6, − 2.7]; p = 0.03). Females without comorbidities also had significantly longer survival compared to all other IPF clusters. There were no significant differences in rate of lung function decline or survival between clusters in the other fibrotic ILD subtypes. Conclusions The combination of male sex and OSA may portend worse outcomes in IPF. Further research is required to elucidate the interplay between sex and comorbidities in ILD, as well as the role of OSA in ILD disease progression.

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