TY - JOUR
TI - Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: Countrywide data in 50 patients
AU - Laskari, K.
AU - Tektonidou, P.M.G.
AU - Katsiari, P.C.
AU - Athanassiou, P.
AU - Dimopoulou, D.
AU - Gerodimos, C.
AU - Salamaliki, C.
AU - Papagoras, P.C.
AU - Settas, P.L.
AU - Vassilopoulos, P.D.
AU - Voulgari, P.P.V.
AU - Zakalka, M.
AU - Georgiadis, A.
AU - Gkoni, G.
AU - Daoussis, P.D.
AU - Dimitroulas, P.T.
AU - Iliou, C.
AU - Kallitsakis, I.
AU - Grika, E.P.
AU - Mavragani, P.C.
AU - Pikazis, P.D.
AU - Raftakis, J.
AU - Sarikoudis, T.
AU - Kougkas, N.
AU - Soukera, D.
AU - Theodorou, E.
AU - Tsatsani, P.
AU - Tsiakou, E.
AU - Vlachoyiannopoulos, P.P.
AU - Vosvotekas, G.
AU - Sfikakis, P.P.P.
JO - Seminars in Arthritis and Rheumatism
PY - 2021
VL - 51
TODO - 1
SP - 137-143
PB - W.B. Saunders
SN - 0049-0172
TODO - 10.1016/j.semarthrit.2020.10.011
TODO - abatacept;  anakinra;  canakinumab;  corticosteroid;  immunoglobulin;  methotrexate;  methylprednisolone;  tocilizumab;  tumor necrosis factor inhibitor;  antirheumatic agent;  biological product;  canakinumab;  monoclonal antibody, adolescent;  adult;  adult onset Still disease;  aged;  Article;  clinical article;  cohort analysis;  disease activity;  disease resistance;  drug dose intensification;  drug efficacy;  drug megadose;  drug safety;  drug withdrawal;  female;  follow up;  genital tract infection;  human;  leukopenia;  longitudinal study;  macrophage activation syndrome;  male;  monotherapy;  mouth infection;  multicenter study;  onset age;  pneumonia;  priority journal;  relapse;  remission;  respiratory tract infection;  retrospective study;  skin infection;  soft tissue infection;  treatment outcome;  treatment response;  urinary tract infection;  clinical trial;  off label drug use;  treatment outcome, Adult;  Antibodies, Monoclonal, Humanized;  Antirheumatic Agents;  Biological Products;  Humans;  Off-Label Use;  Retrospective Studies;  Still's Disease, Adult-Onset;  Treatment Outcome
TODO - Objective: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. Methods:: In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150–300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3–84) months. Results: Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. Conclusion: High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab. © 2020
ER -