Treatment of systemic sclerosis complications: what to use when first-line treatment fails--a consensus of systemic sclerosis experts

Objectives: There is a need for standardization in systemic sclerosis (SSc) management.

Methods: SSc experts (n = 117) were sent 3 surveys to gain consensus for SSc management.

Results: First-line therapy for scleroderma renal crisis (SRC) was an angiotensin-converting enzyme inhibitor (ACEi). For SRC there were not many differences between treating mild or severe SRC. In general, Second-line was to add either a calcium channel blocker (CCB) or angiotensin receptor blocker (ARB) and then an alpha-blocker (66% agreed). Endothelin receptor agonists (ERAs) were the first treatment in mild pulmonary arterial hypertension (PAH) (72%), followed by adding a phosphodiesterase-5 inhibitor (PDE5i) (77%) and then a prostanoid (73%). For severe PAH, initial treatment was 1 of the following: a prostanoid (49%), combination of a ERA and a PDE5i (18%), or combination of a ERA and a prostanoid (16%) (71% agreed). For mild Raynaud's phenomenon (RF), after a CCB and adding a PDE5i (35%), trying an ARB (32%) and finally a prostanoid (23%) was suggested. For more severe RF, 54% agreed on adding a PDE5i (45%) or prostanoid (32%) to a CCB. In the prevention of digital ulcers (DU), initial treatment was a CCB (73%), then adding a PDE5i, then use of a ERA, and then a prostanoid (44% agreed). In interstitial lung disease/pulmonary fibrosis, for induction, usually intravenous cyclophosphamide and mycophenolate mofetil (MMF) or azathioprine were chosen. For maintenance, MMF was chosen by three-fourths (56% agreed). For gastroesophageal reflux disease, >50% would exceed the maximum recommended proton pump inhibitor dose if required (72% agreed). For skin involvement after methotrexate, MMF was usually chosen (37% agreement). For SSC-related inflammatory arthritis, methotrexate therapy (60%) was followed by adding corticosteroids (37%) or hydroxychloroquine (31%) (62% agreed).

Conclusions: Discrepancies in drug choices occurred in treatment after first line in SSc. Not all algorithms had good agreement. This study provides some guidance for SSc management.