Summary
Despite intense research efforts and major advances in the understanding of particular aspects of the disease process, the etiology of SSc is still unknown and the pathogenesis only partly understood. Survival has markedly improved over the past two decades, with the 10-year survival rate approaching 80%, although to date with the exception of cyclophosphamide there is no effective disease-modifying treatment of SSc. The introduction of drugs that treat organ complications has changed markedly, the mortality and life quality of subgroups of SSc. The associated side effects and lack of efficacy in certain subgroups of organ involvement indicate however, that the available treatment options are still unsatisfactory (e.g. in pulmonary fibrosis or kidney failure). Therefore, future treatments should be designed to specifically target distinct pathogenetic pathways in SSc.
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Hunzelmann, N., Krieg, T. (2011). Progressive Systemic Scleroderm. In: Hertl, M. (eds) Autoimmune Diseases of the Skin. Springer, Vienna. https://doi.org/10.1007/978-3-211-99225-8_8
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