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Research ArticleSystemic Sclerosis

Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis

Jeska K. de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic and Alexandra Balbir-Gurman
The Journal of Rheumatology February 2020, 47 (2) 249-254; DOI: https://doi.org/10.3899/jrheum.181173
Jeska K. de Vries-Bouwstra
From Leiden University Medical Center, Department of Rheumatology, Leiden, the Netherlands; Paris Descartes University, Cochin Hospital, Rheumatology Department, Paris, France; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel.
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  • For correspondence: j.k.de_vries-bouwstra@lumc.nl
Yannick Allanore
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Marco Matucci-Cerinic
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Alexandra Balbir-Gurman
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    Table 1.

    Recommendations for treatment of systemic sclerosis; mean level of agreement among SSc experts (n = 209).

    RecommendationsAgreement, Mean (SD)
    CCB should be considered as first-line therapy for SSc-RP8.2 (2.7)
    PDE-5 inhibitors should be considered in treatment of SSc-RP7.3 (2.7)
    Fluoxetine might be considered in treatment of SSc-RP attacks4.6 (2.8)
    Intravenous iloprost should be considered for severe SSc-RP7.9 (2.7)
    Intravenous iloprost should be considered in the treatment of DU in patients with SSc8.7 (2.3)
    PDE-5 inhibitors should be considered in the treatment of DU in patients with SSc8.0 (2.5)
    Bosentan should be considered for reduction of number of new DU in patients with SSc7.9 (2.8)
    ERA should be considered to treat SSc-related PAH*8.8 (2.4)
    PDE-5 inhibitors should be considered to treat SSc-related PAH*8.9 (2.3)
    Riociguat should be considered to treat SSc-related PAH*7.4 (2.8)
    Intravenous epoprostenol should be considered for treatment of patients with severe SSc-related PAH*8.3 (2.5)
    Prostacyclin analogs should be considered to treat SSc-related PAH*8.0 (2.7)
    MTX may be considered for skin manifestations of early diffuse SSc7.4 (2.8)
    Cyclophosphamide should be considered for treatment of SSc-ILD, in particular for patients with progressive ILD8.0 (2.6)
    HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure7.1 (2.9)
    Experts recommend immediate use of ACE inhibitors in the treatment of SRC9.2 (2.1)
    Blood pressure and renal function should be carefully monitored in SSc patients treated with glucocorticoids9.0 (2.2)
    PPI should be used for the treatment of SSc-related GERD, and prevention of esophageal ulcers and strictures9.0 (2.2)
    Prokinetic drugs should be used for the management of SSc-related symptomatic motility disturbances8.0 (2.4)
    Intermittent or rotating use of antibiotics may be useful to treat symptomatic bacterial overgrowth in patients with SSc8.5 (2.1)
    • ↵* N = 166: of n = 209 complete responders, 21% did not complete the questions regarding PAH-specific drugs because they stated that they felt uncomfortable answering questions regarding guidelines on treatment of SSc-related PAH. SSc: systemic sclerosis; CCB: dihydropyridine-type calcium channel blockers; PDE-5: phosphodiestherase type 5; ACE: angiotensin-converting enzyme; PPI: proton pump inhibitors; RP: Raynaud phenomenon; DU: digital ulcers; ERA: endothelin receptor antagonists; PAH: pulmonary arterial hypertension; MTX: methotrexate; ILD: interstitial lung disease; HSCT: hematopoietic stem cells transplant; SRC: scleroderma renal crises; GERD: gastroesophageal reflux disease (nonsignificant).

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    Table 2.

    Treatment recommendations with different agreements between European SSc experts and non-European SSc experts.

    RecommendationsEuropean Experts, n = 157Non-European Experts, n = 63p
    Intravenous iloprost should be considered for severe SSc-RP8.5 (2.2)6.5 (3.1)< 0.0001
      Availability, %9055
    Intravenous iloprost should be considered in the treatment of DU in patients with SSc9.0 (1.9)7.4 (2.7)< 0.0001
      Availability, %9359
    Bosentan should be considered for reduction of number of new DU in patients with SSc8.5 (2.3)6.3 (3.2)< 0.0001
      Availability, %8657
    MTX may be considered for skin manifestations of early diffuse SSc7.9 (2.7)6.8 (3.0)< 0.05
      Availability, %9592
    Cyclophosphamide should be considered for treatment of SSc-ILD, in particular for patients with progressive ILD8.3 (2.4)7.4 (2.9)< 0.05
      Availability, %99100
    HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure7.3 (2.7)6.4 (3.2)< 0.05
      Availability, %6666
    • Values are mean (SD) unless otherwise specified. SSc: systemic sclerosis; RP: Raynaud phenomenon; DU: digital ulcers; MTX: methotrexate; ILD: interstitial lung disease; HSCT: hematopoietic stem cell transplant.

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    Table 3.

    Treatment recommendations with different agreements between experts stratified according to drug/treatment option availability.

    RecommendationsDrug AvailableDrug Not Availablep
    PDE-5 inhibitors should be considered in treatment of SSc-RP7.9 (2.5), n = 1296.4 (2.7), n = 88< 0.0001
    Fluoxetine might be considered in treatment of SSc-RP attacks4.9 (2.9), n = 1384.1 (2.5), n = 81< 0.05
    Intravenous iloprost should be considered for severe SSc-RP8.3 (2.6), n = 1706.8 (2.7), n = 44< 0.001
    Intravenous iloprost should be considered in the treatment of DU in patients with SSc8.8 (2.2), n = 1767.5 (2.6), n = 37< 0.0001
    Bosentan should be considered for reduction of number of new DU in patients with SSc8.3 (2.4), n = 1656.5 (3.0), n = 48< 0.0001
    Riociguat should be considered to treat SSc-related PAH8.1 (2.4), n = 996.6 (3.0), n = 66< 0.0001
    HSCT should be considered for treatment of selected patients with rapidly progressive SSc at risk of organ failure7.4 (2.8), n = 1366.5 (2.8), n = 71< 0.05
    • Values are mean (SD) unless otherwise specified. PDE-5: phosphodiestherase type 5; SSc: systemic sclerosis; RP: Raynaud phenomenon; DU: digital ulcers; PAH: pulmonary arterial hypertension; HSCT: hematopoietic stem cell transplant.

Supplementary Materials

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Additional Files

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Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis
Jeska K. de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic, Alexandra Balbir-Gurman
The Journal of Rheumatology Feb 2020, 47 (2) 249-254; DOI: 10.3899/jrheum.181173

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Worldwide Expert Agreement on Updated Recommendations for the Treatment of Systemic Sclerosis
Jeska K. de Vries-Bouwstra, Yannick Allanore, Marco Matucci-Cerinic, Alexandra Balbir-Gurman
The Journal of Rheumatology Feb 2020, 47 (2) 249-254; DOI: 10.3899/jrheum.181173
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SYSTEMIC SCLEROSIS
DRUG THERAPY
PRACTICE GUIDELINES
GUIDELINE ADHERENCE

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