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. 2021 Dec 22;8:788250. doi: 10.3389/fmed.2021.788250

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Copyright © 2021 Zanin-Silva, Santana-Gonçalves, Kawashima-Vasconcelos and Oliveira.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Mechanistic targets of the main pharmacological approaches for systemic sclerosis-related vasculopathy. Multiple drug options, with different mechanistic approaches, can be used for management of vascular manifestations in SSc patients. This figure summarizes mechanisms and clinical indications for: calcium channel blockers (I); endothelin-1 receptor antagonists (II); phosphodiesterase-5 (PDE-5) inhibitors (III); angiotensin-converting enzyme (ACE) inhibitors (IV); guanylate cyclase stimulator – riociguat (V) and prostanoids (VI). SR: strength of recommendation; SR: A, SR: B or SR: C according to the European League against Rheumatism, in 2017 (87). Dihydropyridines, PDE-5 inhibitors and iloprost are recommended for SSc-associated Raynaud's phenomenon (SR: A); bosentan is recommended to reduce the number of new digital ulcers (SR: A); PDE-5 inhibitors and iloprost are recommended for active digital ulcers (SR: A); PDE-5 inhibitors, endothelin-1 receptor antagonists (bosentan, ambrisentan, and macitentan) and riociguat are recommended for treatment of SSc-related pulmonary artery hypertension (SR: B); epoprostenol (SR: A) and other prostacyclin analogs (SR: B) are recommended for severe SSc-related pulmonary artery hypertension; ACE inhibitors are recommended as treatment for scleroderma renal crisis (SR: C). ^* and ^** indicate clinical conditions in which specific drugs from the class are recommended. NO, nitric oxide; ET-1, endothelin-1; ET_A, type A endothelin receptor; ET_B, type B endothelin receptor; cGMP, cyclic guanosine-5-monophosphate; sGC, soluble guanylate cyclase; cAMP, cyclic adenosine monophosphate; GTP, guanosine-5′-triphosphate; IP, prostacyclin receptor; PGI-2, prostaglandin I-2.