Immune-Targeted Therapies in SSc

This review summarises recent developments in immune-mediated approaches for the management of systemic sclerosis (SSc). We will also discuss potential mechanisms to which these agents that may have on the immunological aspect of the disease process. Current approaches to disease-modifying therapy in SSc together with strategies that are in use to address individual complications of the disease are reviewed in other chapters. Available immunosuppressives only modestly impact the course of the disease. Most drugs are currently only investigated in the subset of patients with early diffuse cutaneous SSc. In this patient population, the significance of immunologically driven disease burden is best exemplified by the benefit derived from hematopoietic stem-cell transplantation as the only treatment that has demonstrated reversal of skin disease, enhanced quality of life, and reduced long-term mortality, but associated with high risks and costs. With an improved understanding of SSc immunopathogenesis we hope that together with the progress made in the design of clinical trials and outcome parameters, identification of novel targets will likely result in the improvement of effective management options.