title: Serum angiopoietin-like protein 3 levels: possible correlation with progressive skin sclerosis, digital ulcers and pulmonary vascular involvement in patients with systemic sclerosis.
abstract: Angiopoietin-like protein 3 (ANGPTL3), which is part of a family of secreted glycoproteins that are structurally similar to angiopoietins, is principally expressed in the liver and is involved in lipid metabolism and angiogenesis. The aim of this study was to determine the clinical significance of serum ANGPTL3 levels, measured with a specific enzyme-linked immunosorbent assay, in patients with systemic sclerosis. Serum ANGPTL3 levels correlated positively with skin score in diffuse cutaneous systemic sclerosis with a disease duration ≤ 6 years. Furthermore, the prevalence of digital ulcers was significantly higher in patients with elevated serum ANGPTL3 levels than in other patients. Moreover, among patients excluding diffuse cutaneous systemic sclerosis with disease duration ≤ 6 years, serum ANGPTL3 levels correlated positively with estimated right ventricular systolic pressure. In conclusion, ANGPTL3 may contribute to the development of progressive skin sclerosis and proliferative obliterative vasculopathy, such as digital ulcers and pulmonary vascular involvement leading to pulmonary arterial hypertension, in systemic sclerosis.
authors.affiliation: Department of Dermatology, University of Tokyo Graduate School of Medicine, Tokyo 113-8655, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Ichimura
authors.forename: Yoshihide
authors.initials: Y
authors.lastname: Asano
authors.forename: Kaname
authors.initials: K
authors.lastname: Akamata
authors.forename: Naohiko
authors.initials: N
authors.lastname: Aozasa
authors.forename: Shinji
authors.initials: S
authors.lastname: Noda
authors.forename: Takashi
authors.initials: T
authors.lastname: Taniguchi
authors.forename: Takehiro
authors.initials: T
authors.lastname: Takahashi
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Toyama
authors.forename: Hayakazu
authors.initials: H
authors.lastname: Sumida
authors.forename: Yosihiro
authors.initials: Y
authors.lastname: Kuwano
authors.forename: Koichi
authors.initials: K
authors.lastname: Yanaba
authors.forename: Yayoi
authors.initials: Y
authors.lastname: Tada
authors.forename: Makoto
authors.initials: M
authors.lastname: Sugaya
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kadono
mesh_terms: D000328:Adult; D000368:Aged; D000091303:Angiopoietin-Like Protein 3; D000075802:Angiopoietin-like Proteins; D042682:Angiopoietins / Q000097:blood*; D001794:Blood Pressure / Q000502:physiology; D016022:Case-Control Studies; D004797:Enzyme-Linked Immunosorbent Assay; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D005385:Fingers / Q000503:physiopathology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000097:blood* / Q000503:physiopathology; D012883:Skin Ulcer / Q000097:blood* / Q000503:physiopathology; D013599:Systole / Q000502:physiology; D016278:Ventricular Function, Right / Q000502:physiology
chemical_list: C479896:ANGPTL3 protein, human; D000091303:Angiopoietin-Like Protein 3; D000075802:Angiopoietin-like Proteins; D042682:Angiopoietins