TY - JOUR
TI - Treatment modalities and drug survival in a systemic sclerosis real-life patient cohort
AU - Panopoulos, S.
AU - Chatzidionysiou, K.
AU - Tektonidou, M.G.
AU - Bournia, V.K.
AU - Drosos, A.A.
AU - Liossis, S.-N.C.
AU - Dimitroulas, T.
AU - Sakkas, L.
AU - Boumpas, D.
AU - Voulgari, P.V.
AU - Daoussis, D.
AU - Thomas, K.
AU - Georgiopoulos, G.
AU - Vosvotekas, G.
AU - Garyfallos, A.
AU - Sidiropoulos, P.
AU - Bertsias, G.
AU - Vassilopoulos, D.
AU - Sfikakis, P.P.
JO - Arthritis Research and Therapy
PY - 2020
VL - 22
TODO - 1
SP - null
PB - BioMed Central Ltd.
SN - null
TODO - 10.1186/s13075-020-2140-3
TODO - 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
TODO - 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).
ER -