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Thyroid Disease Is Prevalent and Predicts Survival in Patients With Idiopathic Pulmonary Fibrosis.

ABSTRACT: BACKGROUND:A significant minority of patients with idiopathic pulmonary fibrosis (IPF) display features of autoimmunity without meeting the criteria for overt connective tissue disease. A link between IPF and other immune-mediated processes, such as hypothyroidism (HT), has not been reported. In this investigation, we aimed to determine whether HT is associated with IPF and if outcomes differ between patients with IPF with and without HT. METHODS:A retrospective case-control analysis was conducted. Of 311 patients referred to the University of Chicago Interstitial Lung Disease Center with an initial diagnosis of IPF, 196 met the inclusion criteria and were included in the final analysis. Each case was matched 1:1 by age, sex, and race to a control subject with COPD. RESULTS:HT was identified in 16.8% of cases and 7.1% of control subjects (OR, 2.7; 95% CI, 1.31-5.54; P = .01). Among patients with IPF, HT was associated with reduced survival time (P < .001) and was found to be an independent predictor of mortality in multivariable Cox regression analysis (hazard ratio, 2.12; 95% CI, 1.31-3.43; P = .002). A secondary analysis of two IPF clinical trial datasets supports these findings. CONCLUSIONS:HT is common among patients with IPF, with a higher prevalence than in those with COPD and the general population. The presence of HT also predicts mortality in IPF, a finding that may improve future prognostication models. More research is needed to determine the biologic link between IPF and HT and how the presence of thyroid disease may influence disease progression.

SUBMITTER: Oldham JM

PROVIDER: S-EPMC4556122 | biostudies-literature | 2015 Sep

REPOSITORIES: biostudies-literature

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Thyroid Disease Is Prevalent and Predicts Survival in Patients With Idiopathic Pulmonary Fibrosis.

Oldham Justin M JM Kumar Disha D Lee Cathryn C Patel Shruti B SB Takahashi-Manns Stephenie S Demchuk Carley C Strek Mary E ME Noth Imre I

Chest 20150901 3

<h4>Background</h4>A significant minority of patients with idiopathic pulmonary fibrosis (IPF) display features of autoimmunity without meeting the criteria for overt connective tissue disease. A link between IPF and other immune-mediated processes, such as hypothyroidism (HT), has not been reported. In this investigation, we aimed to determine whether HT is associated with IPF and if outcomes differ between patients with IPF with and without HT.<h4>Methods</h4>A retrospective case-control analy ...[more]

PMID: 25811599

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Project description:BackgroundA number of genetic markers linked to familial pulmonary fibrosis predict differential survival in interstitial lung disease (ILD) patients. Although genetic testing is not performed routinely for ILD, family history commonly is obtained and may inform outcome risk.Research questionDoes survival vary between patients with and without self-reported familial pulmonary fibrosis?MethodsFamily history was acquired systematically for consecutive ILD patients who consented to clinical registry enrollment at the University of Texas Southwestern and the University of California at Davis. Patients were stratified by idiopathic pulmonary fibrosis (IPF) and non-IPF ILD diagnosis and were substratified by presence or absence of familial pulmonary fibrosis, defined as one or more additional affected family members. Transplant-free survival was compared using multilevel, mixed-effects Cox proportional hazards regression.ResultsOf the 1,262 patients included, 534 (42%) had IPF ILD and 728 (58%) had non-IPF ILD. Of those with non-IPF ILD, 18.5% had connective tissue disease, 15.6% had chronic hypersensitivity pneumonitis, and 23.5% had unclassifiable ILD. Familial pulmonary fibrosis was reported in 134 IPF ILD patients (25.1%) and 90 non-IPF ILD patients (12.4%). Those with familial IPF showed an 80% increased risk of death or transplantation compared with those with sporadic IPF (hazard ratio [HR], 1.8; 95% CI, 1.37-2.37; P < .001), whereas those with familial non-IPF ILD showed a twofold increased risk compared with their counterparts with sporadic disease (HR, 2.08; 95% CI, 1.46-2.96; P < .001). Outcome risk among those with familial non-IPF ILD was no different than for those with sporadic IPF ILD (HR, 1.27; 95% CI, 0.89-1.84; P = .19).InterpretationPatient-reported familial pulmonary fibrosis is predictive of reduced transplant-free survival in IPF and non-IPF ILD patients. Because survival among patients with familial non-IPF ILD approximates that of sporadic IPF ILD, early intervention should be considered for such patients. Until clinical genetic testing is widely available and provides actionable results, family history should be ascertained and considered in risk stratification.

Project description:BackgroundA number of circulating plasma biomarkers have been shown to predict survival in patients with idiopathic pulmonary fibrosis (IPF), but most were identified before the use of antifibrotic (AF) therapy in this population. Because pirfenidone and nintedanib have been shown to slow IPF progression and may prolong survival, the role of such biomarkers in AF-treated patients is unclear.Research questionTo determine whether plasma concentration of cancer antigen 125 (CA-125), C-X-C motif chemokine 13 (CXCL13), matrix metalloproteinase 7 (MMP7), surfactant protein D (SP-D), chitinase-3-like protein-1 (YKL-40), vascular cell adhesion protein-1 (VCAM-1), and osteopontin (OPN) is associated with differential transplant-free survival (TFS) in AF-exposed and nonexposed patients with IPF.Study design and methodsA pooled, multicenter, propensity-matched analysis of IPF patients with and without AF exposure was performed. Optimal thresholds for biomarker dichotomization were identified in each group using iterative Cox regression. Longitudinal biomarker change was assessed in a subset of patients using linear mixed regression modeling. A clinical-molecular signature of IPF TFS was then derived and validated in an independent IPF cohort.ResultsThree hundred twenty-five patients were assessed, of which 68 AF-exposed and 172 nonexposed patients were included after propensity matching. CA-125, CXCL13, MMP7, YKL-40, and OPN predicted differential TFS in AF-exposed patients but at higher thresholds than in AF-nonexposed individuals. Plasma biomarker level generally increased over time in nonexposed patients but remained unchanged in AF-exposed patients. A clinical-molecular signature predicted decreased TFS in AF-exposed patients (hazard ratio [HR], 5.91; 95% CI, 2.25-15.5; P < .001) and maintained this association in an independent AF-exposed cohort (HR, 3.97; 95% CI, 1.62-9.72; P = .003).InterpretationMost plasma biomarkers assessed predicted differential TFS in AF-exposed patients with IPF, but at higher thresholds than in nonexposed patients. A clinical-molecular signature of IPF TFS may provide a reliable predictor of outcome risk in AF-treated patients but requires additional research for optimization and validation.

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Dataset Information

Thyroid Disease Is Prevalent and Predicts Survival in Patients With Idiopathic Pulmonary Fibrosis.

Publications

Thyroid Disease Is Prevalent and Predicts Survival in Patients With Idiopathic Pulmonary Fibrosis.

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