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AB0731 Treatment algorithms for systemic sclerosis according to experts

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Background Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. Objectives To generate SSc treatment algorithms endorsed by high percentage of SSc experts. Methods Experts from the Scleroderma Clinical… Click to show full abstract

Background Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. Objectives To generate SSc treatment algorithms endorsed by high percentage of SSc experts. Methods Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (n=170) were asked whether they agreed with SSc algorithms (from 20121). A further 2 consensus rounds refined agreement; 62 (36%), 54 and 48 experts completed surveys. Results For scleroderma renal crisis (SRC), 82% of the experts agreed (1st line ACEi, 2nd and 3rd adding: CCB or ARB). Pulmonary arterial hypertension (PAH) had 81% agreement. For mild PAH, PDE5i, then endothelin receptor antagonists plus PDE5i, then prostanoids; while for severe PAH prostanoids were first-line. Raynauds’ phenomenon (RP) had 78% of agreement [mild (1st CCB, 2nd adding PDE5i, 3rd ARB or switching to another CCB, 4th prostanoids), severe (1st CCB, 2nd adding PDE5i, 3rd ERA, 4th prostanoids)]. Digital ulcer (DU) treatment had 69% agreement (1st CCB, 2nd PDE5i). Interstitial lung disease (ILD) had 65% agreement including induction (Mycophenolate mofetil (MMF) then intravenous cyclophosphamide then rituximab) and maintenance (1st line MMF). Skin involvement had 71% agreement. For a modified Rodnan skin score (mRSS) of 24 1st MTX, 2nd MMF; and for mRSS 32 1st MMF, 2nd MTX, 3rd intravenous cyclophosphamide (CYP), 4th hematopoietic stem cell transplantation. For inflammatory arthritis 79% agreed with 1st MTX, 2nd low dose glucocorticoids, 3rd hydroxychloroquine, 4th rituximab or tocilizumab. Cardiac and gastrointestinal algorithms had ≥75% agreement. The evolution of the agreement rates is shown in table 1:Abstract AB0731 – Table 1 Algorithms for SSc treatment Agreement 20121 (%) Agreement 2017 (%) Scleroderma renal crisis 69 82 Pulmonary arterial hypertension 45 81 Raynaud’s phenomenon 66 78 Digital ulcers 58 69 Interstitial lung disease 64 65 Gastrointestinal involvement NA 77 Skin involvement 56, 40, 36† 71 Inflammatory arthritis 45 79 Cardiac involvement NA 75 Agreement is the% who agreed to the algorithm. SSc: Systemic Sclerosis; NA: Not applicable; †For modified Rodnan skin scores 10, 24 and 32, respectively Conclusions Total agreement for SSc algorithms was considerable. These SSc algorithms may guide treatment. Reference [1] Walker KM, Pope J. Treatment of Systemic Sclerosis Complications: What to Use When First-Line Treatment Fails-A Consensus of Systemic Sclerosis Experts. Semin Arthritis Rheum2012;42:42–55. Disclosure of Interest A. Fernández-Codina Grant/research support from: Spanish Federation for Internal Medicine; Ontario Scleroderma Association, K. Walker: None declared, J. Pope Grant/research support from: AbbVie, Actelion, Amgen, BMS, GSK, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB, Consultant for: AbbVie, Actelion, Amgen, BMS, GSK, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, UCB

Keywords: agreement; ccb; ssc; treatment; systemic sclerosis

Journal Title: Annals of the Rheumatic Diseases
Year Published: 2018

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