Early systemic sclerosis—opportunities for treatment

Systemic sclerosis (SSc) is characterized by microvasculopathy (Raynaud’s phenomenon and fibrointimal proliferation), presence of autoantibodies and collagen deposition in skin (scleroderma) and internal organs. Microvasculopathy, detected by nailfold capillaroscopy, and disease-specific autoantibod...

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Bibliographic Details
Published in:Clinical rheumatology 2015-08, Vol.34 (8), p.1327-1331
Main Authors: Sakkas, Lazaros I., Simopoulou, Theodora, Katsiari, Christina, Bogdanos, Dimitrios, Chikanza, Ian C.
Format: Article
Language:English
Subjects:
Autoantibodies
Early Diagnosis
Humans
Immunosuppressive Agents - therapeutic use
Medicine
Medicine & Public Health
Microscopic Angioscopy
Prognosis
Raynaud Disease - diagnosis
Review Article
Rheumatology
Scleroderma, Systemic - diagnosis
Scleroderma, Systemic - drug therapy
Scleroderma, Systemic - immunology
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Summary:Systemic sclerosis (SSc) is characterized by microvasculopathy (Raynaud’s phenomenon and fibrointimal proliferation), presence of autoantibodies and collagen deposition in skin (scleroderma) and internal organs. Microvasculopathy, detected by nailfold capillaroscopy, and disease-specific autoantibodies (anti-topoisomerase I, anti-centromere, anti-RNA polymerase III antibodies) usually appear earlier, even years before scleroderma. At that stage of the disease, immune activation with T cells and B cells promote fibrosis. Diagnosis of SSc has been relied on scleroderma, and by this time, internal organs may have developed fibrosis, a lethal feature with no available treatment. The new EULAR/ACR 2013 criteria for the classification of SSc will help identify SSc patients before fibrosis of internal organs. The early diagnosis of SSc, before the development of fibrosis in internal organs, will allow the introduction of immunosuppressive medications in these patients in a controlled setting (randomized trials). It is anticipated that this approach will change the hitherto grim prognosis of SSc for the better.
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-015-2902-5