Conclusion
Although SSc is an uncommon disease, it is important because it has the highest mortality of any of the rheumatic diseases, and also because it is a paradigm for other more common medical conditions in which immunologically triggered fibrosis occurs, such as liver cirrhosis and idiopathic pulmonary fibrosis.
The life-threatening nature of the worst forms of SSc and the lack of treatments of proven efficacy merit careful consideration of the best approaches to treatment. Combination therapy has already established itself by virtue of the need to treat organ-based complications of SSc, and is beginning to transform the long-term outlook in this disease. Although combination approaches also represent the most likely strategies to be effective for disease-modifying treatment, the timing, duration and precise agents needed to achieve this are still being defined. Evaluation of treatments is confounded by the heterogeneity of the disease, its relative rarity and the difficulties associated with objective longitudinal assessment, as outlined above.
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Denton, C.P., Black, C.M. Combination therapies for systemic sclerosis. Springer Semin Immunopathol 23, 109–129 (2001). https://doi.org/10.1007/s002810100058
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DOI: https://doi.org/10.1007/s002810100058