Systemic sclerosis (SSc) is a multi-systemic fibrotic disorder with worldwide distribution and high morbidity and mortality. Characteristic features of this disease are widespread vasculopathy, inflammation, autoimmunity, and fibrosis. The better clinical outcome in recent years is mainly due to better management of organ complications. To date, there is no approved specific therapy to prevent or slow down the overall progression of the disease. So far, conventional disease-modifying antirheumatic drugs (DMARDs) have had no major impact on the disease course and have not prolonged survival. Based on recent studies of molecular pathomechanisms and various animal models, key molecules of fibrogenesis and vasculopathy in SSc could be identified. Therefore, to date, we have to reconsider and redefine the objectives of our treatment strategies. In this article, we discuss current and future therapeutic concepts as well as the objectives of new treatment strategies and of the evaluation of diagnostic tools with respect to pulmonary arterial hypertension, lung fibrosis and skin/systemic fibrosis.

No abstract