Περίληψη:
Background: European 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. Methods: Details 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. Results: Methotrexate 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. Conclusions: Existing 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. © 2020 The Author(s).
Συγγραφείς:
Panopoulos, S.
Chatzidionysiou, K.
Tektonidou, M.G.
Bournia, V.K.
Drosos, A.A.
Liossis, S.-N.C.
Dimitroulas, T.
Sakkas, L.
Boumpas, D.
Voulgari, P.V.
Daoussis, D.
Thomas, K.
Georgiopoulos, G.
Vosvotekas, G.
Garyfallos, A.
Sidiropoulos, P.
Bertsias, G.
Vassilopoulos, D.
Sfikakis, P.P.
Λέξεις-κλειδιά:
azathioprine; calcium channel blocking agent; cyclophosphamide; endothelin receptor antagonist; iloprost; methotrexate; mycophenolic acid; rituximab; sildenafil; tocilizumab; azathioprine; cyclophosphamide; drug; endothelin receptor antagonist; immunosuppressive agent; vasoconstrictor agent, adult; Article; clinical feature; cohort analysis; disease duration; drug efficacy; drug safety; drug withdrawal; female; finger ulcer; human; lung fibrosis; major clinical study; male; middle aged; retrospective study; survival rate; systemic sclerosis; treatment duration; unspecified side effect; aged; classification; systemic sclerosis, Adult; Aged; Azathioprine; Cohort Studies; Cyclophosphamide; Endothelin Receptor Antagonists; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Pharmaceutical Preparations; Retrospective Studies; Scleroderma, Systemic; Vasoconstrictor Agents
