eprintid: 10166289 rev_number: 10 eprint_status: archive userid: 699 dir: disk0/10/16/62/89 datestamp: 2023-03-09 17:34:36 lastmod: 2023-08-29 10:46:04 status_changed: 2023-03-09 17:34:36 type: article metadata_visibility: show sword_depositor: 699 creators_name: Pope, Janet E creators_name: Denton, Christopher P creators_name: Johnson, Sindhu R creators_name: Fernandez-Codina, Andreu creators_name: Hudson, Marie creators_name: Nevskaya, Tatiana title: State-of-the-art evidence in the treatment of systemic sclerosis ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: G90 keywords: Drug therapy, Stem-cell therapies, Systemic sclerosis note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. abstract: Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evident from randomized clinical trials. Treatments for early dcSSc include immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab and tocilizumab. Patients with rapidly progressive early dcSSc might be eligible for autologous haematopoietic stem cell transplantation, which can improve survival. Morbidity from interstitial lung disease and pulmonary arterial hypertension is improving with the use of proven therapies. Mycophenolate mofetil has surpassed cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Nintedanib and possibly perfinidone can be considered in SSc pulmonary fibrosis. Pulmonary arterial hypertension is frequently treated with initial combination therapy (for example, with phosphodiesterase 5 inhibitors and endothelin receptor antagonists) and, if necessary, the addition of a prostacyclin analogue. Raynaud phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers (especially nifedipine), then phosphodiesterase 5 inhibitors or intravenous iloprost. Bosentan can reduce the development of new digital ulcers. Trial data for other manifestations are mostly lacking. Research is needed to develop targeted and highly effective treatments, best practices for organ-specific screening and early intervention, and sensitive outcome measurements. date: 2023-04 date_type: published publisher: Nature Publishing Group official_url: https://doi.org/10.1038/s41584-023-00909-5 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 2009353 doi: 10.1038/s41584-023-00909-5 medium: Print-Electronic pii: 10.1038/s41584-023-00909-5 lyricists_name: Denton, Christopher lyricists_id: CPDEN87 actors_name: Allington-Smith, Dominic actors_id: DAALL44 actors_role: owner full_text_status: public publication: Nature Reviews Rheumatology volume: 19 pagerange: 212-226 event_location: United States issn: 1759-4790 citation: Pope, Janet E; Denton, Christopher P; Johnson, Sindhu R; Fernandez-Codina, Andreu; Hudson, Marie; Nevskaya, Tatiana; (2023) State-of-the-art evidence in the treatment of systemic sclerosis. Nature Reviews Rheumatology , 19 pp. 212-226. 10.1038/s41584-023-00909-5 <https://doi.org/10.1038/s41584-023-00909-5>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10166289/1/Denton_NSS%20SSc%20review%20Pope%20Denton%20et%20al%202023%20for%20UCL.pdf