@article{10902/33815, year = {2024}, url = {https://hdl.handle.net/10902/33815}, abstract = {Background: A substantial proportion of patients with giant cell arteritis (GCA) relapse despite standard therapy with glucocorticoids, methotrexate and tocilizumab. The Janus kinase/signal transducer and activator of transcription (JAK/STAT) signalling pathway is involved in the pathogenesis of GCA and JAK inhibitors (JAKi) could be a therapeutic alternative. We evaluated the effectiveness of JAKi in relapsing GCA patients in a real-world setting and reviewed available literature. Methods: Retrospective analysis of GCA patients treated with JAKi for relapsing disease at thirteen centers in Spain and one center in United States (01/2017-12/2022). Outcomes assessed included clinical remission, complete remission and safety. Clinical remission was defined as the absence of GCA signs and symptoms regardless of the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values. Complete remission was defined as the absence of GCA signs and symptoms along with normal ESR and CRP values. A systematic literature search for other JAKi-treated GCA cases was conducted. Results: Thirty-five patients (86% females, mean age 72.3) with relapsing GCA received JAKi therapy (baricitinib, n=15; tofacitinib, n=10; upadacitinib, n=10). Before JAKi therapy, 22 (63%) patients had received conventional synthetic immunosuppressants (e.g., methotrexate), and 30 (86%) biologics (e.g., tocilizumab). After a median (IQR) follow-up of 11 (6-15.5) months, 20 (57%) patients achieved and maintained clinical remission, 16 (46%) patients achieved and maintained complete remission, and 15 (43%) patients discontinued the initial JAKi due to relapse (n=11 [31%]) or serious adverse events (n=4 [11%]). A literature search identified another 36 JAKi-treated GCA cases with clinical improvement reported for the majority of them. Conclusions: This real-world analysis and literature review suggest that JAKi could be effective in GCA, including in patients failing established glucocorticoid-sparing therapies such as tocilizumab and methotrexate. A phase III randomized controlled trial of upadacitinib is currently ongoing (ClinicalTrials.gov ID NCT03725202).}, organization = {Funding: No specific funding was received from any bodies in the public, commercial or non-for-profit sectors to carry out the work described in this article. Acknowledgements: We thank all the members of the different hospitals and patients included in this study.}, publisher = {BioMed Central}, publisher = {Arthritis Research & Therapy, 2024, 26, 116}, title = {Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature}, author = {Loricera García, Javier and Tofade, Toluwalase and Prieto Peña, Diana and Romero-Yuste, Susana and Miguel, Eugenio de and Riveros-Frutos, Anne and Ferraz-Amaro, Iván and Labrador, Eztizen and Maiz, Olga and Becerra, Elena and Narváez, Javier and Galíndez-Agirregoikoa, Eva and González-Fernández, Ismael and Urruticoechea-Arana, Ana and Ramos-Calvo, Ángel and López-Gutiérrez, Fernando and Castañeda, Santos and Unizony, Sebastián and Blanco Alonso, Ricardo}, }