Background: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive dyspnea and worsening lung function due to remodeling of the lung, including epithelial mesenchymal transition. ADAM33 is a disintegrin and metalloprotease domain-containing protein, which may be related to lung fibrosis by exerting angiogenesis and remodeling of the lung. Thus, we evaluated the association of single-nucleotide polymorphisms (SNPs) of ADAM33 with the risk of IPF.
Methods: A total of 237 patients with IPF and 183 healthy subjects participated in the present study. Nine polymorphisms were selected. Genotyping was performed by single-base extension. Polymorphisms and haplotypes were analyzed for associations with the risk of IPF using multiple logistic regression models controlling for age, gender, and smoking status as covariates.
Results: All SNPs were in Hardy-Weinberg equilibrium. The minor allele frequency (MAF) of rs628977G>A in intron 21 was significantly lower in subjects with surgical IPF than in normal controls in the recessive model [33.2 vs. 38.0 %, p = 0.02, OR = 0.40 (0.19-0.84)]. When the subjects with clinical IPF were included, the difference in MAF persisted with a p value of 0.03 [OR = 0.50 (0.27-0.94)].
Conclusions: ADAM33 rs628977G>A was marginally associated with a decreased risk of IPF in a recessive model.
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http://dx.doi.org/10.1007/s00408-014-9578-5 | DOI Listing |
Rheumatology (Oxford)
January 2025
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
J Thorac Dis
December 2024
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Background: The benefits of pulmonary rehabilitation (PR) for patients with idiopathic pulmonary fibrosis (IPF) have been limited to improving dyspnea, exercise capacity, and quality of life (QoL). This study aimed to assess the current status of PR and its effect on prognosis.
Methods: The Nationwide Korean Health Insurance Review and Assessment Service (HIRA) database was used in this study.
J Thorac Dis
December 2024
Division of Pulmonary, Allergy and Critical Care Medicine, Department of Internal Medicine, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Background: Patients with idiopathic pulmonary fibrosis (IPF) are at risk of lung cancer development. Antifibrotic therapy could slow disease progression of IPF, but there is limited data on its effectiveness on lung cancer. Here, we aimed to investigate lung cancer incidence and the risk of mortality of patients with IPF receiving antifibrotic therapy.
View Article and Find Full Text PDFJ Thorac Dis
December 2024
Department of Respiratory Medicine, the First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Zhengzhou, China.
Background: Idiopathic pulmonary fibrosis (IPF) has high mortality and poor prognosis, which brings enormous burdens to families and society. We conducted this meta-analysis to analyze and summarize the risk factors associated with mortality in IPF, hoping to provide reference for clinical prevention and treatment of IPF.
Methods: We conducted a comprehensive search of PubMed, Cochrane Library, Embase, and Web of Science from inception to August 10, 2023, to include cohort studies on mortality in patients with IPF.
Cell Rep
January 2025
School of Life Science and Technology, ShanghaiTech University, Shanghai 201210, China; State Key Laboratory of Advanced Medical Materials and Devices, ShanghaiTech University, Shanghai 201210, China. Electronic address:
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease characterized by excess accumulation of the extracellular matrix (ECM). The role of macrophage-fibroblast crosstalk in lung fibrogenesis is incompletely understood. Here we found that fibroblast growth factor-inducible molecule 14 (Fn14), the receptor for tumor necrosis factor-like weak inducer of apoptosis (TWEAK) is highly induced in myofibroblasts in the lungs of IPF patients and the bleomycin-induced lung fibrosis model.
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