title: Recent advances in understanding the pathogenesis of scleroderma-interstitial lung disease.
abstract: Systemic sclerosis (scleroderma, SSc) is a heterogeneous autoimmune connective tissue disease of unknown etiology. Interstitial lung disease (ILD) is a frequent complication, and a significant contributor to morbidity and mortality among SSc patients. SSc-ILD most commonly occurs within 10 years of diagnosis, and may be seen in patients with either the limited or diffuse cutaneous subset of SSc. SSc-ILD is a multifaceted disease process in which different factors and pathways are involved. Aberrant function of a variety of lung cells, cytokines, growth factors, peptides, and bioactive proteins, in combination with genetic and epigenetic regulators, have crucial functions in the pathogenesis of this disease. Here we present our view on recent advances regarding the pathogenesis of SSc-ILD.
authors: Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, 114 Doughty Street, Charleston, SC, 29425, USA, akter@musc.edu.
authors: Tanjina
authors: T
authors: Akter
authors: Richard M
authors: RM
authors: Silver
authors: Galina S
authors: GS
authors: Bogatkevich
mesh_terms: D057890:Epigenomics; D020022:Genetic Predisposition to Disease; D006681:HLA-D Antigens / Q000235:genetics; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000235:genetics / Q000473:pathology; D013917:Thrombin / Q000502:physiology; D016212:Transforming Growth Factor beta / Q000502:physiology; D051176:beta Catenin / Q000502:physiology
chemical_list: C495270:CTNNB1 protein, human; D006681:HLA-D Antigens; D016212:Transforming Growth Factor beta; D051176:beta Catenin; D013917:Thrombin