Treatment Algorithms for Systemic Sclerosis According to Experts

Arthritis Rheumatol. 2018 Nov;70(11):1820-1828. doi: 10.1002/art.40560. Epub 2018 Sep 17.


Objective: There is a lack of agreement regarding treatment for many aspects of systemic sclerosis (SSc). We undertook this study to generate SSc treatment algorithms endorsed by a 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, 54, and 48 experts (36%, 32%, and 28%, respectively) completed the first, second, and third surveys, respectively.

Results: For treatment of scleroderma renal crisis, 81% of experts agreed (first-, second-, and third-line treatments were angiotensin-converting enzyme inhibitors, then adding calcium-channel blockers [CCBs], then adding angiotensin receptor blockers [ARBs], respectively). For pulmonary arterial hypertension (PAH), 81% of experts agreed (for mild PAH, treatments were phosphodiesterase 5 [PDE5] inhibitors, then endothelin receptor antagonists plus PDE5 inhibitors, then prostanoids, respectively; for severe PAH, prostanoids were first-line treatment). For mild Raynaud's phenomenon (RP), 79% of experts agreed (treatments were CCBs, then adding PDE5 inhibitors, then ARBs or switching to another CCB, respectively; after the third line of treatment, mild RP was deemed severe). For severe RP, the first- through fourth-line treatments were CCBs, then adding PDE5 inhibitors or prostanoids, then adding PDE5 inhibitors (if not added as second-line treatment) or prostanoids (if not added as second-line treatment), then switching to another CCB, respectively. For active treatment of digital ulcers, 66% of experts agreed (first- and second-line treatments were CCBs and PDE5 inhibitors, respectively). For interstitial lung disease, 69% of experts agreed (for induction therapy, treatments were mycophenolate mofetil [MMF], intravenous cyclophosphamide [IV CYC], and rituximab, respectively; for maintenance, first-line treatment was MMF). For skin involvement, 71% of experts agreed (for a modified Rodnan skin thickness score [MRSS] of 24, first- and second-line treatments were methotrexate [MTX] and MMF, respectively; for an MRSS of 32, first- through fourth-line treatments were MMF, MTX, IV CYC, and hematopoietic stem cell transplantation, respectively). For inflammatory arthritis, 79% of experts agreed (first- through fourth-line treatments were MTX, low-dose glucocorticoids, hydroxychloroquine, and rituximab or tocilizumab, respectively). Algorithms for cardiac and gastrointestinal involvement had ≥75% agreement.

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

Publication types

  • Consensus Development Conference
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Algorithms*
  • Angiotensin Receptor Antagonists / therapeutic use
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Antirheumatic Agents / therapeutic use
  • Arthritis / drug therapy*
  • Arthritis / etiology
  • Calcium Channels / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Endothelin Receptor Antagonists / therapeutic use
  • Glucocorticoids / therapeutic use
  • Humans
  • Hydroxychloroquine
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology
  • Immunosuppressive Agents / therapeutic use
  • Kidney Diseases / drug therapy*
  • Kidney Diseases / etiology
  • Lung Diseases, Interstitial / drug therapy*
  • Lung Diseases, Interstitial / etiology
  • Methotrexate / therapeutic use
  • Mycophenolic Acid / therapeutic use
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Prostaglandins / therapeutic use
  • Raynaud Disease / drug therapy*
  • Raynaud Disease / etiology
  • Rituximab / therapeutic use
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / drug therapy*


  • Angiotensin Receptor Antagonists
  • Angiotensin-Converting Enzyme Inhibitors
  • Antibodies, Monoclonal, Humanized
  • Antirheumatic Agents
  • Calcium Channels
  • Endothelin Receptor Antagonists
  • Glucocorticoids
  • Immunosuppressive Agents
  • Phosphodiesterase 5 Inhibitors
  • Prostaglandins
  • Rituximab
  • Hydroxychloroquine
  • Cyclophosphamide
  • Mycophenolic Acid
  • tocilizumab
  • Methotrexate