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Systemic sclerosis (SSc) is a rare and complex disease, involving multiple organs, with high morbidity and mortality. Fibrosis is the hallmark of SSc, although vascular and inflammatory mechanisms are also implicated in its pathogenesis. Disease management is challenging, due to its heterogeneous presentation, and to the limited number of controlled clinical trials to guide treating clinicians. Immunosuppressive agents have been used to prevent progression, especially in the lung, before irreversible injury occurs, with some, although mo