Comorbidities in unclassifiable interstitial lung disease

Prior, Thomas Skovhus; Hyldgaard, Charlotte; Torrisi, Sebastiano Emanuele; Kronborg-White, Sissel; Ganter, C.; Bendstrup, Elisabeth; Kreuter, Michael

doi:10.1186/s12931-022-01981-3

Cited by 7 publications

(6 citation statements)

References 28 publications

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“…In patients with IPF, four clusters of comorbidities have been reported [8] and in unclassi able ILD, three comorbidity clusters were identi ed. [19] Similar to our results, a cluster of patients with few comorbidities and a cluster dominated by cardiovascular diseases and male patients were found in both cohorts. This supports the robustness of these clusters.…”

Section: Discussionsupporting

confidence: 91%

Clusters of comorbidities in fibrotic hypersensitivity pneumonitis

Prior

Wälscher

Gross

et al. 2022

Preprint

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Background: Hypersensitivity pneumonitis (HP) is a type of interstitial lung disease (ILD) with a variable disease course and prognosis ranging from inflammatory and self-limiting to irreversible and progressive pulmonary fibrosis. Comorbidities are common in HP and may have an impact on prognosis. Due to the heterogeneity of HP presentation and progression, the identification of specific phenotypes in relationship to disease course and outcome is essential. The aim of this study was to identify clusters of comorbidities which could represent phenotypes in fibrotic HP and examine their impact on prognosis. Methods: Patients diagnosed with fibrotic HP at a tertiary referral center for ILD were included. Comorbidities were systematically registered and clusters of comorbidities were identified using cluster analyses. Disease progression and survival was estimated for each cluster.Results: The cohort comprised 211 patients with 53.6% males, mean age 63.0, baseline FVC 72.7%, DLCO 44.1%. Median follow-up time was 1.8 years (IQR 0.7-3.9). Three clusters with distinct comorbidity profiles and clinical characteristics were identified. One cluster dominated by elder male patients with predominantly cardiovascular diseases was associated with more respiratory hospitalizations and a worse prognosis. Differences in pulmonary function or exercise capacity trajectories between clusters were not observed.Conclusions: Three clusters with distinct comorbidities were identified and could represent phenotypes in fibrotic HP not previously recognized. The worst prognosis was observed in a cluster dominated by elder males with cardiovascular diseases. Increased focus on prevention and treatment of comorbidities could potentially improve the prognosis of patients with fibrotic HP.

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Section: Discussionsupporting

confidence: 91%

Clusters of comorbidities in fibrotic hypersensitivity pneumonitis

Prior

Wälscher

Gross

et al. 2022

Preprint

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“…This was consistent with a study by Oldham, et al, where GERD was the most prevalent (52.8%) comorbidity in an IPAF cohort raising suspicion for a potential contribution of GERD to disease pathogenesis in IPAF (3). In addition, COPD, depression, and DM were found to be common comorbid conditions in our cohort, a nding which was not discussed in prior IPAF literature but has been demonstrated in other forms of ILD (12,30,31).…”

Section: Discussionsupporting

confidence: 54%

Evaluation of Comorbidity Burden on Disease Progression and Mortality in Patients with Interstitial Pneumonia with Autoimmune Features: a Retrospective Cohort Study

Joerns,

Ghebranious,

Adams

et al. 2023

Preprint

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Background Interstitial pneumonia with autoimmune features (IPAF) is a subset of interstitial lung disease that manifests with interstitial pneumonia and features of autoimmunity while not meeting classification criteria for a defined rheumatic disease. Comorbidity burden is an important prognostic indicator in various rheumatic and interstitial lung diseases, but few studies have commented on comorbidities in this population. This study was conducted to evaluate the association of individual comorbidities, the Charlson Comorbidity Index (CCI), and the Rheumatic Disease Comorbidity Index (RDCI) with lung disease progression and transplant/mortality outcomes in patients with IPAF. Methods In a retrospective study, we evaluated the prevalence and severity of comorbidities in an institutional cohort of patients with IPAF. Using Cox regression, we correlated the association of individual comorbidities and comorbidity burden using CCI and RDCI with time to lung disease progression (defined as relative forced vital capacity (FVC) decline of 10% or more) and with time to lung transplant/all-cause mortality. We compared the performance of CCI and RDCI, while adjusting for the Interstitial Lung Disease Gender-Age-Physiology (ILD-GAP) index. Results In a sample of 201 individuals with IPAF, a history of cerebrovascular accident (CVA) or cardiovascular disease (CVD), moderate to severe chronic kidney disease, or fracture was associated with a faster onset of lung disease progression, while a history of gastroesophageal reflux was protective. History of CVA/CVD, diabetes mellitus, and lymphoma were associated with a faster onset of lung transplant/death. Both CCI and RDCI were significantly associated with shorter time to lung disease progression (hazard ratio [HR] 1.11, 95% confidence interval [CI] 1.04–1.19 and HR 1.12 with 95%CI 1.00-1.26, respectively) and lung transplant/mortality (HR 1.18 [1.07–1.30] and 1.31 [1.10–1.57], respectively). Conclusions CCI and RDCI may be useful tools in assessing prognosis in patients with IPAF in terms of both lung disease progression and mortality. Prospective studies are needed to further evaluate the performance of CCI and RDCI and the impact of optimizing comorbid conditions that may mitigate poor outcomes among patients with IPAF.

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“…Third, our study is focused on differences across race/ethnic groups, complex social constructs that often reflect an individual's perception of their familial origin, cultural environment, and genetic makeup 37 . Fourth, it is also possible that the observed association of short telomeres with a detrimental survival pattern is due to mortality from comorbidities in PF, including coronary artery disease, pulmonary hypertension, dyslipidemia, combined pulmonary fibrosis and emphysema, obstructive sleep apnea, gastro-esophageal reflux disease, among others [38][39][40] . Given that physiologic age (linked to short telomeres) may be more important than chronological age in driving mortality due to comorbidities, statistical adjustments for age would not necessarily account for these.…”

Section: Discussionmentioning

confidence: 99%

Telomere length associates with chronological age and mortality across racially diverse pulmonary fibrosis cohorts

et al. 2023

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Pulmonary fibrosis (PF) is characterized by profound scarring and poor survival. We investigated the association of leukocyte telomere length (LTL) with chronological age and mortality across racially diverse PF cohorts. LTL measurements among participants with PF stratified by race/ethnicity were assessed in relation to age and all-cause mortality, and compared to controls. Generalized linear models were used to evaluate the age-LTL relationship, Cox proportional hazards models were used for hazard ratio estimation, and the Cochran–Armitage test was used to assess quartiles of LTL. Standardized LTL shortened with increasing chronological age; this association in controls was strengthened in PF (R = −0.28; P < 0.0001). In PF, age- and sex-adjusted LTL below the median consistently predicted worse mortality across all racial groups (White, HR = 2.21, 95% CI = 1.79–2.72; Black, HR = 2.22, 95% CI = 1.05–4.66; Hispanic, HR = 3.40, 95% CI = 1.88–6.14; and Asian, HR = 2.11, 95% CI = 0.55–8.23). LTL associates uniformly with chronological age and is a biomarker predictive of mortality in PF across racial groups.

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Comorbidities in unclassifiable interstitial lung disease

Cited by 7 publications

References 28 publications

Clusters of comorbidities in fibrotic hypersensitivity pneumonitis

Clusters of comorbidities in fibrotic hypersensitivity pneumonitis

Evaluation of Comorbidity Burden on Disease Progression and Mortality in Patients with Interstitial Pneumonia with Autoimmune Features: a Retrospective Cohort Study

Telomere length associates with chronological age and mortality across racially diverse pulmonary fibrosis cohorts

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