title: Use of biologics and other novel therapies for the treatment of systemic sclerosis.
abstract: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation and fibrosis. These three main disease-determining pathways are the target of the currently available treatments used to possibly modify the progression of disease-related manifestations, although this synergy has not been fully applied on SSc joint, skin or lung involvement yet. Areas covered: we describe the current status of SSc treatment/therapy performing a literature search in MEDLINE/Pubmed and Thomson Reuter's Web of Science for articles published until March 2016. Moreover, ongoing registered clinical trials (RCTs) on SSc were searched through clinicaltrials.gov website. Expert commentary: presently, promising drugs are under evaluation to target the different pathogenic pathways of systemic sclerosis: Tocilizumab and Abatacept for skin and lung fibrosis; Riociguat and Selexipag are approved for pulmonary arterial hypertension but promising anti-fibrotic effects are now being studied. Finally, several anti-fibrotic molecules are currently involved in RCTs, such as Nintedanib, IVA-337, Terguride.
authors.affiliation: a Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC , University of Firenze , Firenze , Italy.
authors.forename: Cosimo
authors.initials: C
authors.lastname: Bruni
authors.affiliation: a Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC , University of Firenze , Firenze , Italy.|b Interdisciplinary Dept of Medicine, division of Rheumatology , University of Bari , Bari , Italy.
authors.forename: Emanuela
authors.initials: E
authors.lastname: Praino
authors.affiliation: c Department of Rheumatology A and INSERM U1016 , Paris Descartes University, Cochin Hospital , Paris , France.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.affiliation: d Department of Rheumatology , University Hospital Zurich , Zurich , Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.affiliation: e Department Of Clinical and Molecular Sciences, Clinica Medica , University 'Politecnica delle Marche' , Ancona , Italy.
authors.forename: Armando
authors.initials: A
authors.lastname: Gabrielli
authors.affiliation: b Interdisciplinary Dept of Medicine, division of Rheumatology , University of Bari , Bari , Italy.
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.affiliation: a Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC , University of Firenze , Firenze , Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci-Cerinic
mesh_terms: D000069594:Abatacept / Q000627:therapeutic use; D000081:Acetamides / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001688:Biological Products / Q000627:therapeutic use*; D002986:Clinical Trials as Topic; D005343:Fibrinolytic Agents / Q000627:therapeutic use*; D005355:Fibrosis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D007211:Indoles / Q000627:therapeutic use; D007249:Inflammation; D007596:Joints / Q000473:pathology*; D008090:Lisuride / Q000031:analogs & derivatives / Q000627:therapeutic use; D008168:Lung / Q000473:pathology*; D039781:PubMed; D011719:Pyrazines / Q000627:therapeutic use; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D012867:Skin / Q000473:pathology*
chemical_list: D000081:Acetamides; D061067:Antibodies, Monoclonal, Humanized; D001688:Biological Products; D005343:Fibrinolytic Agents; D007211:Indoles; D011719:Pyrazines; D011720:Pyrazoles; D011743:Pyrimidines; C006208:dironyl; C523468:selexipag; D000069594:Abatacept; D008090:Lisuride; C530716:nintedanib; C502936:tocilizumab; C542595:riociguat
keywords: Systemic sclerosis; biologics; fibrosis; immunity; inflammation; novel therapies; vasculopathy