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Frustration may be the word that best characterizes many scleroderma management plans - owing to a lack of trials, lack of agreement and lack of clear guidance on management.
A panel of concerned experts on systemic sclerosis (SSc) set out to develop consensus on SSc treatment algorithms.
Experts were from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (n = 170). Consensus was reached in several areas:
- scleroderma renal crisis: 81% of experts agreed that first‐, second‐, and third‐line treatments were angiotensin‐converting enzyme inhibitors, then adding calcium‐channel blockers [CCBs], then adding angiotensin receptor blockers [ARBs], respectively).
- 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).
- mild Raynaud's phenomenon: 79% of experts agreed (treatments were CCBs, then adding PDE5 inhibitors, then ARBs or switching to another CCB, respectively;
- Severe Raynauds: 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).
- 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).
- Skin involvementt: 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).
- Inflammatory arthritis:79% of experts agreed (first‐ through fourth‐line treatments were MTX, low‐dose glucocorticoids, hydroxychloroquine, and rituximab or tocilizumab, respectively).