Systemic sclerosis: An update in 2016

被引:133
作者
Desbois, Anne Claire [1 ,2 ,3 ,4 ]
Cacoub, Patrice [1 ,2 ,5 ]
机构
[1] Univ Paris 06, Sorbonne Univ, UMR 7211, Paris, France
[2] Inflammat Immunopathol Biotherapy Dept DHU i2B, F-75005 Paris, France
[3] Univ Paris 06, INSERM, UMR S 959, F-75013 Paris, France
[4] CNRS, FRE3632, F-75005 Paris, France
[5] Grp Hosp Pitie Salpetriere, AP HP, Dept Internal Med & Clin Immunol, 83 Blvd Hop, F-75013 Paris, France
关键词
Systemic sclerosis; Autoimmune disease; Treatment; PULMONARY ARTERIAL-HYPERTENSION; INTERSTITIAL LUNG-DISEASE; RNA-POLYMERASE-III; STEM-CELL TRANSPLANTATION; SCLERODERMA RENAL CRISIS; GENOME-WIDE ASSOCIATION; SKIN THICKNESS SCORE; GENETIC RISK-FACTOR; RAYNAUDS-PHENOMENON; MYCOPHENOLATE-MOFETIL;
D O I
10.1016/j.autrev.2016.01.007
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
071005 [微生物学]; 100108 [医学免疫学];
摘要
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. (C) 2016 Published by Elsevier B.V.
引用
收藏
页码:417 / 426
页数:10
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