Fig. 1.

Treatment recommendations in SSc

Expert consensus and a growing evidence base underpin the management of SSc, as shown schematically with focus on individual organ systems and current approaches used in practice. MSK: musculoskeletal; ILD: interstitial lung disease; PAH: pulmonary arterial hypertension; RCT: randomized controlled trial; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase-5 inhibitor; sGC: soluble guanylate cyclase; CCBs: calcium channel blockers; ARB: angiotensin receptor blocker; DU: digital ulcer; SRC: scleroderma renal crisis; ACEi: angiotensin converting enzyme inhibitor.