TY - JOUR T1 - Anakinra in Patients With Systemic Juvenile Idiopathic Arthritis: Long-term Safety From the Pharmachild Registry JF - The Journal of Rheumatology JO - J Rheumatol SP - 398 LP - 407 DO - 10.3899/jrheum.210563 VL - 49 IS - 4 AU - Gabriella Giancane AU - Riccardo Papa AU - Sebastiaan Vastert AU - Francesca Bagnasco AU - Joost F. Swart AU - Pierre Quartier AU - Jordi Antón AU - Sylvia Kamphuis AU - Helga Sanner AU - Mia Glerup AU - Fabrizio De Benedetti AU - Elena Tsitsami AU - Agustin Remesal AU - Estefania Moreno AU - Jaime De Inocencio AU - Charlotte Myrup AU - Chiara Pallotti AU - Isabelle Koné-Paut AU - Karin Franck-Larsson AU - Håkan Malmström AU - Susanna Cederholm AU - Angela Pistorio AU - Nico Wulffraat AU - Nicolino Ruperto Y1 - 2022/04/01 UR - http://www.jrheum.org/content/49/4/398.abstract N2 - Objective To evaluate the long-term safety profile of anakinra in patients with systemic juvenile idiopathic arthritis (sJIA).Methods Data from patients with sJIA enrolled in the Pharmachild registry (ClinicalTrials.gov: NCT03932344) prior to September 30, 2018, and treated with anakinra were analyzed. The study endpoints were the occurrence of non-serious adverse events (SAEs) of at least moderate severity and SAEs, including macrophage activation syndrome (MAS), and the duration of anakinra treatment with reasons for discontinuation. All endpoints were analyzed overall by 6-month time windows, and in different treatment sets represented by those patients treated continuously with anakinra for at least 12, 18, and 24 months (set-12, -18, and -24, respectively).Results Three hundred six patients were enrolled. Of these patients, 46%, 34%, and 28% had been treated for at least 12, 18, and 24 months, respectively. Two hundred and one AEs, mostly represented by infections, were reported for 509.3 patient-years (PY) with an overall incidence rate (IR) of 39.5 per 100 PY. Among 56 SAEs (IR 11.0/100 PY), 23.2% were infections and 19.6% MAS episodes. The IR of AEs was higher during the first 6 months of anakinra treatment, followed by decreasing IRs in the long-term treatment sets. Treatment discontinuation occurred in 76% of patients, most frequently in the first 6 months, because of inefficacy (43%), remission (31%), or AEs/intolerance (15%). No deaths or malignancies occurred during anakinra treatment.Conclusion The results of the present study confirm the long-term safety profile of anakinra in patients with sJIA and demonstrate an overall decreasing incidence of AEs over time. [ClinicalTrials.gov: NCT01399281 and NCT03932344] ER -