Abstract
BackgroundEuropean data indicate that systemic sclerosis (SSc)-related death rates are increasing, thus raising concerns about SSc’s optimal management. Herein, we describe current treatment modalities and drug survival in a real-life SSc cohort.MethodsDetails on immunosuppressive/antiproliferative (methotrexate, mycophenolate, cyclophosphamide, azathioprine, rituximab, tocilizumab) and vasoactive agent [(endothelin receptor antagonists (ERAs), sildenafil, iloprost, and calcium channel blockers (CCB)] administration during the disease course (11.8 ± 8.4 years, mean + SD) of 497 consecutive patients examined between 2016 and 2018 were retrospectively recorded. Drug survival was assessed by Kaplan–Meier analysis.ResultsMethotrexate was the most frequently administered immunosuppressive/antiproliferative agent (53% of patients), followed by cyclophosphamide (26%), mycophenolate (12%), and azathioprine (11%). Regarding vasoactive agents, CCB had been ever administered in 68%, ERAs in 38%, iloprost in 7%, and sildenafil in 7% of patients; 23% of patients with pulmonary fibrosis had never received immunosuppressive/antiproliferative agents, 33% of those with digital ulcers had never received ERAs, iloprost, or sildenafil, whereas 19% of all patients had never received either immunosuppressive/antiproliferative or other than CCB vasoactive agents. Survival rates of methotrexate, cyclophosphamide, and mycophenolate differed significantly, being 84/75%, 59/43%, and 74/63% at 12/24 months, respectively, with inefficacy being the most frequent discontinuation cause. Conversely, CCB, ERAs, and sildenafil had high and comparable retention rates of 97/91%, 88/86%, and 80/80%, respectively.ConclusionsExisting therapeutic limitations indicate that more evidence-based treatment is warranted for successful management of SSc. Vasculopathy seems to be managed more rigorously, but the low retention rates of immunosuppressive/antiproliferative drugs suggest that effective and targeted disease-modifying agents are warranted.
Highlights
European data indicate that systemic sclerosis (SSc)-related death rates are increasing, raising concerns about SSc’s optimal management
A study from the EUSTAR group reported that despite a decrease in standardized mortality rate in SSc over time, the rate of deaths directly attributed to SSc has increased [6]
The analysis in the multicentre cohort revealed that MTX was the most commonly administered immunosuppressive/antiproliferative agent in SSc patients followed by CYC, mycophenolate mofetil (MMF), and AZA while a small number of patients was treated with biologics (RTX or TCZ) for which observational studies have reported promising results from their use in SSc [8,9,10,11]
Summary
European data indicate that systemic sclerosis (SSc)-related death rates are increasing, raising concerns about SSc’s optimal management. A study from the EUSTAR group reported that despite a decrease in standardized mortality rate in SSc over time, the rate of deaths directly attributed to SSc has increased [6]. Overall, this lack of improved survival raises concerns about the efficacy of currently available treatments and conveys the need for new, more effective agents. The objectives of the present study are to describe current treatment modalities in a large, multicentre, real-life SSc cohort and to determine the drug survival rate of commonly used immunosuppressive/antiproliferative or vasoactive agents in SSc that reflects their real-world effectiveness and safety
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