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Treatment algorithms for systemic sclerosis according to experts

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Fernández-Codina A1,2, Walker KM3, Pope JE1; Scleroderma Algorithm Group. Collaborators (47) Hughes M, van den Hoogen FH, Vonk M, Valentini G, Tafazzul-E-Haque M, Proudman SM, Mayes MD, Baron M, Orzechowski N, Simeón-Aznar CP, Francesca I, Hsu V, Seibold JR, Steen V, Murdaca G, Walker UA, Silver RM, Pauling JD, Larché M, Khalidi N, Merkel PA, Koenig M, Lally T, Gordon J, Fortin PR, Hayat S, Masetto A, Jacobsen S, Walker J, Denton C, Hesselstrand R, Gabrielli A, Wigley FM, Hunzelmann N, Castelino FV, Douglas Smith C, Johnson SR, Farge D, Boin F, Chung L, Furst DE, Clements P, Vacca A, Riccieri V, Shah A, Inanc M, Nevskaya T. Arthritis Rheumatol. 2018 May 21. doi: 10.1002/art.40560. [Epub ahead of print]


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1 Rheumatology Division, Department of Medicine, University of Western Ontario, London, ON, Canada.

2 Systemic Autoimmune Diseases Unit, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.

3 Rheumatology Division, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada.


INTRODUCTION: 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 2012). Two consensus rounds refined agreement; 62 (36%), 54 (32%) and 48 (28%) experts completed surveys.

RESULTS: For scleroderma renal crisis, 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 (CYP) 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 CYP, 4th hematopoietic stem cell transplantation. For inflammatory arthritis 79% agreed with 1st MTX, 2nd low dose glucocorticoids, 3rdhydroxychloroquine, 4th rituximab or tocilizumab. Cardiac and gastrointestinal algorithms had >75% agreement.

CONCLUSIONS: Total agreement for SSc algorithms was considerable. These SSc algorithms may guide treatment.