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FRI0493 (2020)
THE INTERLEUKIN-1B INHIBITOR CANAKINUMAB FOR REFRACTORY STILL’S DISEASE: LONG-TERM EXPERIENCE IN 50 CONSECUTIVE PATIENTS
K. Laskari1, P. Athanassiou2, A. Georgiadis3, C. Gerodimos4, G. Gkoni5, D. Daoussis6, T. Dimitroulas7, D. Dimopoulou7, C. Iliou8, I. Kallitsakis9, D. Karamitsos10, C. Katsiari11, S. N. Liossis6, C. Mavragani1, C. Papagoras12, D. Pikazis1, I. Raftakis13, T. Sarikoudis14, L. Settas15, P. Sidiropoulos16, D. Soukera17, E. Theodorou18, P. Tsatsani19, E. Tsiakou20, D. Vassilopoulos1, P. Vlachoyiannopoulos1, G. Vosvotekas21, P. V. Voulgari22, M. Zakalka15, M. Tektonidou1, P. Sfikakis1
1Joint Rheumatology Program, NKUA Medical School, Athens, Greece
2Dept. of Rheumatology, St. Paul’s Hospital, Thessaloniki, Greece
3Private Rheumatologist, Ioannina, Greece
4Private Rheumatologist, Volos, Greece
5Rheumatologist, Metropolitan Hospital, Athens, Greece
6Dept. of Rheumatology, University of Patras, Patras, Greece
74th Dept. of Internal Medicine, Aristotle University, Thessaloniki, Greece
8Private rheumatologist, Kozani, Greece
9Private rheumatologist, Chania, Greece
10Rheumatology Clinic, Evangelismos Hospital, Athens, Greece
11Dept. of Rheumatology, Thessaly University, Larissa, Greece
12Private rheumatologist, Alexandroupoli, Greece
13Rheumatology Clinic, Asklepion Hospital, Athens, Greece
14Private Rheumatologist, Kavala, Greece
15First Dept. of Internal Medicine, Rheumatology Section, Aristotle University, Thessaloniki, Greece
16Dept. of Rheumatology, Clinical Immunology and Allergy, University of Crete, Heraklion, Greece
17Private Rheumatologist, Corinth, Greece
18Dept. of Rheumatology, 251 Air Force General Hospital, Athens, Greece
19Dept. of Rheumatology, KAT General Hospital, Athens, Greece
20Private Rheumatologist, Patras, Greece
21Private Rheumatologist, Thessaloniki, Greece
22Rheumatology Clinic, University of Ioannina, Ioannina, Greece

Background: Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Still’s disease and an established therapeutic target.


Objectives: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in adolescent and adult patients with refractory Still’s disease.


Methods: We conducted a retrospective longitudinal outcome study of 50 consecutive patients aged 39 years (median, range 14-72), fulfilling the Yamaguchi disease classification criteria, with active disease despite treatment with corticosteroids (CS) (n=11) and/or methotrexate (n=9) and/or biologics (n=30) [tumor necrosis factor inhibitors (n=13), IL-6 blockade (n=7), abatacept (n=2), anakinra (n=24); ≥1 biologics (n=13)]. Canakinumab 150-300 mg was administered sc, starting every 4 (n=48) or 8 weeks (n=2), for a median of 24 months (range 3-84). Concomitant treatment included CS (n=41), methotrexate (n=12) and leflunomide (n=3).


Results: Complete remission was initially achieved in 78% of patients within a median time of 3 months, irrespective of age at disease onset. Partial clinical and laboratory response was evident in 20%. Canakinumab was discontinued in one patient with resistant disease (primary failure) and in 6 out of 10 initial responders, who relapsed during treatment (secondary failure). Of 39 patients in complete remission, increase in drug administration interval and/or drug dose reduction was attempted in 7, of which only 1 relapsed, whereas drug discontinuation was attempted in 19 patients for a median time of 8 months (range 3-68), of which 8 relapsed. Overall, in half of all disease flares, canakinumab re-introduction or intensification was successful. Canakinumab had a significant CS sparing effect permitting weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient.


Conclusion: In this largest so far real-life patient cohort with refractory Still’s disease, high rates of sustained remission were induced by canakinumab both in adolescent and adult patients.


Disclosure of Interests: Katerina Laskari: None declared, Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Athanasios Georgiadis: None declared, Charalampos Gerodimos: None declared, Georgia Gkoni: None declared, Dimitrios Daoussis: None declared, Theodoros Dimitroulas: None declared, Despoina Dimopoulou: None declared, Chrysoula Iliou: None declared, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Dimitrios Karamitsos: None declared, Christina Katsiari: None declared, Stamatis-Nick Liossis: None declared, Clio Mavragani: None declared, CHARALAMPOS PAPAGORAS: None declared, Dimitrios Pikazis: None declared, Ioannis Raftakis: None declared, Theodosios Sarikoudis: None declared, Loukas Settas: None declared, Prodromos Sidiropoulos: None declared, Despoina Soukera: None declared, Evangelos Theodorou: None declared, Panagiota Tsatsani: None declared, Eleni Tsiakou: None declared, Dimitrios Vassilopoulos: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Georgios Vosvotekas: None declared, Paraskevi V. Voulgari: None declared, Marina Zakalka: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB

Citation: Ann Rheum Dis, volume 79, supplement 1, year 2020, page 841
Session: Other orphan diseases (Poster Presentations)