@article{3000233,
    title = "Treatment modalities and drug survival in a systemic sclerosis real-life patient cohort",
    author = "Panopoulos, S. and Chatzidionysiou, K. and Tektonidou, M.G. and Bournia, V.K. and Drosos, A.A. and Liossis, S.-N.C. and Dimitroulas, T. and Sakkas, L. and Boumpas, D. and Voulgari, P.V. and Daoussis, D. and Thomas, K. and Georgiopoulos, G. and Vosvotekas, G. and Garyfallos, A. and Sidiropoulos, P. and Bertsias, G. and Vassilopoulos, D. and Sfikakis, P.P.",
    journal = "Arthritis Research and Therapy",
    year = "2020",
    volume = "22",
    number = "1",
    publisher = "BioMed Central Ltd.",
    doi = "10.1186/s13075-020-2140-3",
    keywords = "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",
    abstract = "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)."
}