The purpose of this study is to investigate the effectiveness and safety of riociguat (BAY 63-2521) in patients with diffuse cutaneous systemic sclerosis. It is approved by the FDA for the treatment of 2 forms of hypertension in lung blood vessels, chronic thromboembolic pulmonary hypertension (CTEPH) and pulmonary arterial hypertension (PAH). Riociguat works by stimulating an enzyme in cells, which increases the production of another molecule. The resulting effects are a decrease in tissue fibrosis and relaxation of smooth muscle cells (cells found in particular internal organs and blood vessels). It is proposed that riociguat may offer protection against worsening diffuse cutaneous systemic sclerosis.
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