title: Systemic sclerosis: An update in 2016.
abstract: Systemic sclerosis (SSc) is a chronic immune disorder of unknown origin, dominated by excessive fibrosis responsible for cutaneous and pulmonary fibrosis, and by vascular endothelial dysfunction at the origin of skin ischemia, renal and pulmonary artery lesions. Renal and pulmonary complications are mainly responsible for the severity of the disease. Recent advances led to a better understanding of pathological mechanisms and a more accurate classification of patients according to clinical and biological (auto-antibodies) phenotype. Recent trials provided interesting data on different therapeutic strategies, depending on organ involvement. These data are of particular importance in such disease, still characterized by increased mortality and morbidity rates. In this review, we aim to synthetize recent advances in diagnosis and prognosis leading to better classification of SSc patients, and in therapeutic management.
authors.affiliation: Sorbonne Universités, UPMC Université Paris 06, UMR 7211, Paris, France; Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005 Paris, France; INSERM, UMR_S 959, F-75013 Paris, France; CNRS, FRE3632, F-75005 Paris, France.
authors.forename: Anne Claire
authors.initials: AC
authors.lastname: Desbois
authors.affiliation: Sorbonne Universités, UPMC Université Paris 06, UMR 7211, Paris, France; Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005 Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, 83 boulevard de l'hôpital, F-75013 Paris, France. Electronic address: patrice.cacoub@aphp.fr.
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D015415:Biomarkers / Q000032:analysis; D006801:Humans; D008171:Lung Diseases / Q000150:complications; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000628:therapy
chemical_list: D015415:Biomarkers
keywords: Autoimmune disease; Systemic sclerosis; Treatment