川口 鎮司
   Department   School of Medicine(Tokyo Women's Medical University Hospital), School of Medicine
   Position   Clinical Professor
Article types Original article
Language English
Peer review Non peer reviewed
Title Genetic Susceptibility to Interstitial Lung Disease Associated with Systemic Sclerosis.
Journal Formal name:Clinical medicine insights. Circulatory, respiratory and pulmonary medicine
Abbreviation:Clin Med Insights Circ Respir Pulm Med
ISSN code:(1179-5484)1179-5484(Linking)
Domestic / ForeginForegin
Volume, Issue, Page 9(Suppl 1),pp.135-40
Author and coauthor Tochimoto Akiko†, Kawaguchi Yasushi*, Yamanaka Hisashi
Authorship Corresponding author
Publication date 2015
Summary Systemic sclerosis (SSc) is a connective tissue disease that is characterized by tissue fibrosis, microvasculopathy, and autoimmunity. Interstitial lung disease (ILD) is a common complication of SSc and is one of the frequent causes of mortality in SSc. Although the exact etiology of SSc remains unknown, clinical and experimental investigations have suggested that genetic and environmental factors are relevant to the pathogenesis of SSc and SSc-ILD. More than 30 genes have been identified as susceptibility loci for SSc, most of which are involved in immune regulation and inflammation. It is thought that the key pathogenesis of SSc-ILD is caused by the release of profibrotic mediators such as transforming growth factor β1 and connective tissue growth factor from lung cells induced by a persistent damage. This review presents the genetic susceptibility to SSc-ILD, including human leukocyte antigen and non-human leukocyte antigen genes, especially focusing on connective tissue growth factor.
DOI 10.4137/CCRPM.S23312
PMID 26997879