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BACKGROUND Patients with interstitial lung disease (ILD) may have features of an autoimmune disorder that do not meet the diagnostic criteria for connective tissue diseases. We determined the prevalence and characteristics of autoimmune-featured ILD (AIF-ILD) and compared these with those of idiopathic pulmonary fibrosis (IPF) and known connective tissue(More)
Churg-Strauss syndrome is a rare diffuse vasculitis that is almost invariably accompanied by severe asthma. Although overall prognosis is good, and treatment with prednisone alone or in combination with immunosuppressive drugs is usually successful, severe asthma typically persists. Diffuse organ involvement of Churg-Strauss syndrome, especially(More)
We aimed to identify peripheral blood mononuclear cell (PBMC) gene expression profiles predictive of poor outcomes in idiopathic pulmonary fibrosis (IPF) by performing microarray experiments of PBMCs in discovery and replication cohorts of IPF patients. Microarray analyses identified 52 genes associated with transplant-free survival (TFS) in the discovery(More)
BACKGROUND Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease that often causes disabling dyspnea. In IPF and other lung diseases, patient-reported outcomes (PROs)-questionnaires designed to gather information from the patient's perspective-can determine whether therapies affect dyspnea or other outcomes meaningful to patients.(More)
BACKGROUND Even though the pathogenesis of idiopathic pulmonary fibrosis (IPF) is unknown, there is mounting evidence that abnormal reflux (GERD) and aspiration of gastric contents may play a role in the pathogenesis of this disease. AIMS The aims of this study were to determine in patients with GERD and IPF: (a) the clinical presentation, (b) the(More)
Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause or cure, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3-5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients.(More)
BACKGROUND Short telomere lengths are found in a subset of patients with idiopathic pulmonary fibrosis, but their clinical significance is unknown. Our aim was to investigate whether patients with various blood leucocyte telomere lengths had different overall survival. METHODS In this observational cohort study, we enrolled patients with interstitial lung(More)
To the Editor: Idiopathic pulmonary fibrosis is a complex genetic disease; mutations in surfactant protein C, telomerase, and surfactant protein A2 have been identified in familial cases of pulmonary fibrosis.1,2 We confirm a recent association with rs35705950 in the putative promoter of MUC5B and sporadic idiopathic pulmonary fibrosis.3 Patients with(More)
BACKGROUND Idiopathic pulmonary fibrosis (IPF) is a devastating disease that probably involves several genetic loci. Several rare genetic variants and one common single nucleotide polymorphism (SNP) of MUC5B have been associated with the disease. Our aim was to identify additional common variants associated with susceptibility and ultimately mortality in(More)
IMPORTANCE Current prediction models of mortality in idiopathic pulmonary fibrosis (IPF), which are based on clinical and physiological parameters, have modest value in predicting which patients will progress. In addition to the potential for improving prognostic models, identifying genetic and molecular features that are associated with IPF mortality may(More)