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Editorial
Treatment of systemic sclerosis: is there any hope for the future?
  1. Yannick Allanore1,
  2. Marco Matucci-Cerinic2 and
  3. Oliver Distler3
  1. 1Department of Rheumatology A, Cochin Hospital, Paris Descartes University, Paris, France
  2. 2Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy
  3. 3Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  1. Correspondence to Professor Yannick Allanore; yannick.allanore{at}me.com

https://doi.org/10.1136/rmdopen-2016-000260

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    Systemic sclerosis (SSc) is an orphan connective tissue disease characterised by skin and multiorgan involvement. The following original pathological processes distinguish SSc from other connective tissue diseases: (1) microvascular modifications, initially functional and partly reversible, (2) perivascular inflammation which appears to be moderate and perhaps transitory, (3) autoimmune activation, leading to the production of specific and persistent autoantibodies and (4) fibroblast activation, producing an excess of extracellular matrix leading to fibrosis.1 Therefore, SSc is a complex disease with the implication of multiple players in its pathogenesis.

    SSc is a major medical challenge with high mortality and morbidity. In a meta-analysis, the pooled standardised mortality ratio (SMR) was measured as 3.53 (95% CI 3.03 to 4.11) and adjusted metaregression did not show significant changes in SMR over time.2 Nevertheless, some reports did suggest an improved survival in recent years. In an Italian study, the 10-year survival showed a clear-cut increase (81%) compared with older series (69%) from the same centre.3 However, the discrepancy may not only come from improved therapeutic management, but might be due to some changes in the natural history, to an earlier referral of the patients and, even more likely, to the better recognition of patients with milder disease. Accordingly, in the recent Italian series, there were more patients with the limited cutaneous SSc (LcSSc) than in the older study (87.5% vs 72%).3 In terms of organ involvement and progression of the disease, the outcomes in LcSSc and diffuse cutaneous SSc (DcSSc) are different. The new classification criteria will more easily identify patients with LcSSc.4 Therefore, it will become core to require the cutaneous subsetting for any scientific work on SSc and, beyond skin, subclassification or clustering is awaited to improve SSc patient risk stratification. Regarding morbidity, accumulating evidence has shown the huge …

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