The Emerging Targeted Treatments for Giant Cell Arteritis: Beyond Interleukin-6 Inhibition- A Systematic Literature Review
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Abstract
Giant cell arteritis is a type of granulomatous vasculitis that primarily affects the large vessels, particularly the aortic arch and its main and distal branches, in individuals over the age of 50. The treatment of this condition usually necessitates the use of steroid-sparing agents. Among the traditional immunosuppressive medications, methotrexate is the most commonly used, and some research also indicates that leflunomide may be effective. With the introduction of biologic therapies, new treatment options have become available for managing giant cell arteritis. Although the results with tumor necrosis factor alpha inhibitors have been underwhelming in this condition, the inhibition of interleukin 6 through tocilizumab has proven to be a safe and effective treatment choice for giant cell arteritis. This systematic literature review seeks to compile and evaluate all existing clinical evidence regarding the efficacy and safety of biologic disease-modifying antirheumatic drugs that operate through mechanisms other than tumor necrosis factor alpha and interleukin-6 inhibition (including costimulation modulation, interleukin-1 inhibition, interleukin 12/23 inhibition, interleukin 17 inhibition, interleukin 23 inhibition, granulocyte-monocyte colony-stimulating factor inhibition, and B cell depletion) as well as targeted synthetic disease-modifying antirheumatic drugs, such as Janus kinase inhibitors, in patients diagnosed with giant cell arteritis.
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How to Cite
TORUN, Ege Sinan.
The Emerging Targeted Treatments for Giant Cell Arteritis: Beyond Interleukin-6 Inhibition- A Systematic Literature Review.
Medical Research Archives, [S.l.], v. 13, n. 8, aug. 2025.
ISSN 2375-1924.
Available at: <https://esmed.org/MRA/mra/article/view/6850>. Date accessed: 09 nov. 2025.
doi: https://doi.org/10.18103/mra.v13i8.6850.
Section
Review Articles
The Medical Research Archives grants authors the right to publish and reproduce the unrevised contribution in whole or in part at any time and in any form for any scholarly non-commercial purpose with the condition that all publications of the contribution include a full citation to the journal as published by the Medical Research Archives.
References
1. Thiel J. Giant cell arteritis - New treatment targets at the horizon. Semin Arthritis Rheum. 2025;72S:152686.
doi: 10.1016/j.semarthrit.2025.152686
2. Hoffman GS. Giant Cell Arteritis. Ann Intern Med. 2016;165(9):ITC65-ITC80.
doi: 10.7326/AITC201611010
3. Cho HJ, Bloomberg J, Nichols J. Giant cell arteritis. Dis Mon. 2017;63(3):88-91.
doi: 10.1016/j.disamonth.2016.10.006
4. Watelet B, Samson M, de Boysson H, Bienvenu B. Treatment of giant-cell arteritis, a literature review. Mod Rheumatol. 2017;27(5):747-754. doi: 10.1080/14397595.2016.1266070
5. Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J et al. The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time. Semin Arthritis Rheum. 2022 ;55:152010. doi: 10.1016/j.semarthrit.2022.152010
6. Wilson JC, Sarsour K, Collinson N, Tuckwell K, Musselman D et al. Incidence of outcomes potentially associated with corticosteroid therapy in patients with giant cell arteritis. Semin Arthritis Rheum. 2017;46(5):650-656. doi: 10.1016/j.semarthrit.2016.10.001
7. Wilson JC, Sarsour K, Collinson N, Tuckwell K, Musselman D et al. Serious adverse effects associated with glucocorticoid therapy in patients with giant cell arteritis (GCA): A nested case-control analysis. Semin Arthritis Rheum. 2017;46(6):819-827. doi: 10.1016/j.semarthrit.2016.11.006
8. Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016;315(22):2442-58. doi: 10.1001/jama.2016.5444
9. Jover JA, Herna´ndez-Garcı´a C, Morado IC, Vargas E, Bañares A et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone. a randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2001;134:106–14.
10. Koster MJ, Yeruva K, Crowson CS, Muratore F, Labarca C et al. Efficacy of methotrexate in real-world management of giant cell arteritis: a case-control study. J Rheumatol. 2019;46: 501–8.
11. Leon L, Rodriguez-Rodriguez L, Morado I, Rosales Z, Vadillo C et al. Treatment with methotrexate and risk of relapses in patients with giant cell arteritis in clinical practice. Clin Exp Rheumatol. 2018;36:121–8.
12. Kramariˇ c J, Rotar ˇ Z, Tomˇ siˇ c M, Hoˇ cevar A. Performance of leflunomide as a steroid-sparing agent in giant cell arteritis: a single-center, open-label study. Front Med. 2022;9. https://doi.org/10.3389/fmed.2022.1069013
13. Hoˇ cevar A, Jeˇ se R, Rotar ˇ Z, Tomˇ siˇ c M. Does leflunomide have a role in giant cell arteritis? An open-label study. Clin Rheumatol. 2019;38(2):291–6. http://doi.org/10.1007/s10067-018-4232-x
14. Hoffman GS, Cid MC, Rendt-Zagar KE, Merkel PA, Weyand CM et al. Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med. 2007;146:621–30.
15. Seror R, Baron G, Hachulla E, Debandt M, Larroche M et al. Adalimumab for steroid sparing in patients with giant-cell arteritis: results of a multicentre randomised controlled trial. Ann Rheum Dis. 2014;73:2074–81.
16. Martı´nez-Taboada VM, Rodrı´guez-Valverde V, Carreno~ L, López-Longo J, Figueroa M, et al. A double-blind placebo controlled trial of etanercept in patients with giant cell arteritis and corticosteroid side effects. Ann Rheum Dis. 2008;67:625–30.
17. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377:317–28.
18. Villiger PM, Adler S, Kuchen S, Wermelinger F, Dan D et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebocontrolled trial. Lancet. 2016;387:1921–7.
19. European Medicines Agency. RoActemra tocilizumab new indication. Available from, https://www.ema.europa.eu/en/documents/smop/chmp-post-authorisation-summary-positive-opinion-roactemra-ii-66_en.pdf; 2023
20. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377(4):317–28. https://doi.org/10.1056/NEJMoa1613849
21. Samson M, Dasgupta B, Sammel AM, Salvarani C, Pagnoux C et al. Targeting interleukin-6 pathways in giant cell arteritis management: A narrative review of evidence. Autoimmun Rev. 2025 Jan;24(2):103716. doi: 10.1016/j.autrev.2024.103716
22. Schmidt WA, Dasgupta B, Luqmani R, Unizony SH, Blockmans D, et al. A multicentre, randomised, doubleblind, placebo-controlled, parallel-group study to evaluate the efficacy and safety of Sirukumab in the treatment of Giant cell arteritis. Rheumatol Ther. 2020;7(4): 793–810. https://doi.org/10.1007/s40744-020-00227-2
23. A Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Sarilumab in Patients With Giant Cell Arteritis. ClinicalTrials.gov identifier: NCT03600805. Updated 15 March 2022. Accessed 24 June 2025. https://clinicaltrials.gov/study/NCT03600805
24. Pombo-Suarez M, Gomez-Reino JJ. Abatacept for the treatment of rheumatoid arthritis. Expert Rev Clin Immunol. 2019;15(4):319-326. doi: 10.1080/1744666X.2019.1579642
25. Abatacept for Treating Adults With Giant Cell Arteritis and Takayasu's Arteritis. ClinicalTrials.gov identifier: NCT00556439. Updated January 25, 2018. Accessed 24 June 2025. https://clinicaltrials.gov/study/NCT00556439
26. A Study to Evaluate Efficacy and Safety of Subcutaneous Abatacept With Steroid Treatment Compared to Steroid Treatment Alone in Adults With Giant Cell Arteritis (GCA). ClinicalTrials.gov identifier: NCT03192969. Updated July 10, 2017. Accessed 26 June 2025. https://clinicaltrials.gov/study/NCT03192969
27. Abatacept for the Treatment of Giant Cell Arteritis. ClinicalTrials.gov identifier: NCT04474847. Updated 10 February, 2025. Accessed 27 June 2025. https://clinicaltrials.gov/study/NCT04474847
28. Langford CA, Cuthbertson D, Ytterberg SR, Khalidi N, Monach PA,et al. A Randomized, Double-Blind Trial of Abatacept (CTLA-4Ig) for the Treatment of Giant Cell Arteritis. Arthritis Rheumatol. 2017;69(4): 837-845. doi: 10.1002/art.40044
29. Gérard AL, Simon-Tillaux N, Yordanov Y, Cacoub P, Tubach F et al. Efficacy and safety of steroid-sparing treatments in giant cell arteritis according to the glucocorticoids tapering regimen: A systematic review and meta-analysis. Eur J Intern Med. 2021;88:96-103. doi: 10.1016/j.ejim.2021. 03.04
30. Rossi D, Cecchi I, Sciascia S, Naretto C, Alpa M et al. An agent-to-agent real life comparison study of tocilizumab versus abatacept in giant cell arteritis. Clin Exp Rheumatol. 2021;39 Suppl 129(2):125-128. doi: 10.55563/clinexprheumatol/l0hd9v
31. Caton MT Jr, Mark IT, Narsinh KH, Baker A, Cooke DL et al. Endovascular Therapy for Intracranial Giant Cell Arteritis : Systematic Review, Technical Considerations and the Effect of Intra-arterial Calcium Channel Blockers. Clin Neuroradiol. 2022;32(4):1045-1056. doi: 10.1007/s00062-022-01171-0
32. Arnold DD, Yalamanoglu A, Boyman O. Systematic Review of Safety and Efficacy of IL-1-Targeted Biologics in Treating Immune-Mediated Disorders. Front Immunol. 2022;13:888392. doi: 10.3389/fimmu.2022.888392
33. Giant Cell Arteritis and Anakinra Trial (GiAnT). ClinicalTrials.gov identifier: NCT02902731. Updated 21 February 2021. Accessed 28 June 2025. https://clinicaltrials.gov/study/NCT02902731
34. De Boysson H, Ly KH, Geffray L, Quemeneur T, Liozon E et al. Four months of treatment with anakinra combined with glucocorticoids for giant cell arteritis: a multicenter, randomized, double-blind, placebo-controlled trial. Arthritis Res Ther. 2025;27(1):122. doi: 10.1186/s13075-025-03493-z
35. Deshayes S, Ly KH, Rieu V, Maigné G, Martin Silva N et al. Steroid-sparing effect of anakinra in giant-cell arteritis: a case series with clinical, biological and iconographic long-term assessments. Rheumatology (Oxford). 2021;61(1):400-406. doi: 10.1093/rheumatology/keab280
36. Ly KH, Stirnemann J, Liozon E, Michel M, Fain O et al. Interleukin-1 blockade in refractory giant cell arteritis. Joint Bone Spine. 2014;81(1):76-8. doi: 10.1016/j.jbspin.2013.06.004
37. Gallou S, Agard C, Dumont A, Deshayes S, Boutemy J et al. Evolution and outcomes of aortic dilations in giant cell arteritis. Eur J Intern Med. 2024;129:71-77. doi: 10.1016/j.ejim.2024.03.038
38. Dumont A, Parienti JJ, Delmas C, Boutemy J, Maigné G et al. Factors Associated with Relapse and Dependence on Glucocorticoids in Giant Cell Arteritis. J Rheumatol. 2020;47(1):108-116. doi: 10.3899/jrheum.181127
39. Rossi-Semerano L, Fautrel B, Wendling D, Hachulla E, Galeotti C et al. Tolerance and efficacy of off-label anti-interleukin-1 treatments in France: a nationwide survey. Orphanet J Rare Dis. 2015;10:19. doi: 10.1186/s13023-015-0228-7
40. De Boysson H, Liozon E, Espitia O, Daumas A, Vautier M et al. Different patterns and specific outcomes of large-vessel involvements in giant cell arteritis. J Autoimmun. 2019;103:102283. doi: 10.1016/j. jaut.2019. 05.011
41. A randomised, double-blind, placebo-controlled proof-of concept study of the efficacy and safety of gevokizumab in the treatment of patients with giant cell arteritis. EudraCT number: 2013-002778-38 Updated 4 December 2016. Accessed 28 June 2025.
42. Andel PM, Diamantopoulos AP, Myklebust G, Haugeberg G. Vasculitis distribution and clinical characteristics in giant cell arteritis: a retrospective study using the new 2022 ACR/EULAR classification criteria. Front Med (Lausanne). 2023;10:1286601. doi: 10.3389/fmed.2023.1286
43. Roberts J, O'Rielly DD, Rahman P. A review of ustekinumab in the treatment of psoriatic arthritis. Immunotherapy. 2018;10(5):361-372. doi: 10.2217/imt-2017-0149
44. Ustekinumab for the Treatment of Giant Cell Arteritis (UGCA) ClinicalTrials.gov identifier: NCT02955147. Updated 10 June 2020. Accessed 7 July 2025. https://clinicaltrials.gov/study/NCT02955147
45. Matza MA, Fernandes AD, Stone JH, Unizony SH. Ustekinumab for the treatment of Giant cell arteritis. Arthritis Care Res. 2021;73:893–7. https://doi.org/10.1002/ acr.24200
46. Ustekinumab for the Treatment of Relapse of Refractory Giant Cell Arteritis (ULTRA). ClinicalTrials.gov identifier: NCT03711448. Updated 3 October 2024. Accessed 7 July 2025. https://clinicaltrials.gov/study/NCT03711448
47. Conway R, O’Neill L, O’Flynn E, Gallagher P, McCarthy GM et al. Ustekinumab for the treatment of refractory giant cell arteritis. Ann Rheum Dis. 2016;75:1578–9
48. Conway R, O’Neill L, Gallagher P, McCarthy GM, Murphy CC et al. Ustekinumab for refractory giant cell arteritis: A prospective 52-week trial. Semin Arthritis Rheum. 2018;48:523–8.
49. Samson M, Ghesquière T, Berthier S, Bonnotte B. Ustekinumab inhibits Th1 and Th17 polarisation in a patient with giant cell arteritis. Ann Rheum Dis. 2018;77:e6. doi: 10.1136/annrheumdis-2017-211622
50. Abdalla A, Ali I, Murphy D, Molloy E. Connecting the dots: a story of unknown fever, acute coronary syndrome and pan-aortitis-an occult relapse of a large vessel vasculitis. BMJ Case Rep. 2019;12(5):e230424. doi: 10.1136/bcr-2019
51. Maillet F, Nguyen Y, Espitia O, Perard L, Salvarani C et al. Association between large vessel vasculitis and inflammatory bowel disease: a case-control study. Rheumatology(Oxford). 2025;64(6):3724-3732. doi: 10.1093/rheumatology/keaf030
52. Sandler RD, Smith R, Kitsanta P, Hughes M. Paradoxical New Diagnosis of Giant Cell Arteritis While Receiving Ustekinumab. J Clin Rheumatol. 2020;26(7):e215-e216. doi: 10.1097/RHU.0000000000001084
53. Samson M, Bonnotte B. Ustekinumab fo the Treatment of Giant Cell Arteritis: Comment on the Articls by Matza et al. Arthritis Care Res. 2021;73:1058-1059.
54. Conway R, Molloy ES. Ustekinumab in Giant Cell Arteritis: Comment on the Article by Matza et al. Arthritis Care Res. 2021;73:1056–1057.
55. Fauny M, Moulin D, D'Amico F, Netter P, Petitpain N et al. Paradoxical gastrointestinal effects of interleukin-17 blockers. Ann Rheum Dis. 2020;79(9):1132-1138. doi: 10.1136/annrheumdis-2020-217927
56. Study to Evaluate the Pharmacokinetics, Safety and Tolerability of Intravenous Secukinumab in Patients With GCA or PMR ClinicalTrials.gov identifier: NCT06130540. Updated 3 June 2025. Accessed 8 July 2025. https://clinicaltrials.gov/study/NCT06130540
57. A Placebo-controlled Phase 2 Trial to Investigate the Safety and Efficacy of Secukinumab in Giant Cell Arteritis (TitAIN). ClinicalTrials.gov identifier: NCT03765788. Updated 16 August 2023. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT03765788
58. Venhoff N, Schmidt WA, Lamprecht P, Tony HP, App C et al. Efficacy and safety of secukinumab in patients with giant cell arteritis: study protocol for a randomized, parallel group, double-blind, placebo-controlled phase II trial. Trials.2021;22 (1):543. doi: 10.1186/s13063-021-05520-1
59. Venhoff N, Schmidt WA, Bergner R, Rech J, Unger L et al. Safety and efficacy of secukinumab in patients with giant cell arteritis (TitAIN): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Rheumatol. 2023;5(6):e341–e50. doi: 10.1016/S2665- 9913(23)00101-7
60. Phase III Study of Efficacy and Safety of Secukinumab Versus Placebo, in Combination With Glucocorticoid Taper Regimen, in Patients With Giant Cell Arteritis (GCA) (GCAptAIN). ClinicalTrials.gov identifier: NCT04930094. Updated 6 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT04930094
61. Efficacy and Safety of Secukinumab in Patients With New Onset of Giant Cell Arteritis Who Are in Clinical Remission (GigAINt). ClinicalTrials.gov identifier: NCT05380453. Updated 27 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT05380453
62. Tomelleri A, Bond M, Marvisi C, Campochiaro C, Farina N et al. Secukinumab is effective and safe for patients with giant cell arteritis after tocilizumab failure. Rheumatology (Oxford). 2025:keaf250. doi: 10.1093/rheumatology/keaf250
63. Rotar ˇZ, Tomˇsiˇc M, Hoˇcevar A. Secukinumab for the maintenance of glucocorticoid-free remission in a patient with giant cell arteritis and psoriatic arthritis. Rheumatology. 2018;57:934–6. https://doi.org/10.1093/rheumatology/kex507
64. Sammut L, Litwic A, Smith R, Bartram S. 002 Biopsy proven giant cell arteritis in a patient with psoriatic arthritis on TNF-alpha inhibitor treated with high dose prednisolone and a switch to secukinumab (anti IL-17). Rheumatology. 2018;57(suppl_3):key075. 226.
65. Tomelleri A, Rinaldi E, Campochiaro C, Picchio M, Dagna L. Successful use of ixekizumab for glucocorticoid-free remission maintenance in giant cell arteritis. Rheumatology (Oxford). 2023;62(2):e24-e26. doi: 10.1093/rheumatology/ keac416
66. Ghoreschi K, Balato A, Enerbäck C, Sabat R. Therapeutics targeting the IL-23 and IL-17 pathway in psoriasis. Lancet. 2021;397(10275):754-766. doi: 10.1016/S0140-6736(21)00184-7
67. A Study to Evaluate Guselkumab for the Treatment of Participants With New-onset or Relapsing Giant Cell Arteritis (THEIA). Clinical Trials.gov identifier: NCT04633447. Updated 26 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT04633447
68. Crotti C, Biggioggero M, Becciolini A, Agape E, Favalli EG. Mavrilimumab: a unique insight and update on the current status in the treatment of rheumatoid arthritis. Expert Opin Investig Drugs. 2019;28(7):573-581. doi: 10.1080/13543784. 2019.1631795
69. A Phase 2, Randomized, Double-blind Placebo- controlled Study to Test the Efficacy and Safety of KPL-301 in Giant Cell Arteritis. Clinical Trials.gov identifier: NCT03827018. Updated 17 October 2023. Accessed 9 July 2025. http://clinicaltrials.gov/study/NCT0382701
70. Pupim L, Unizony S, Cid M, Pilipski L, Gandhi R et al. A Phase 2, Randomized, Double-Blind Placebo-Controlled Study to Test The Efficacy and Safety of Mavrilimumab in Giant Cell Arteritis: Study Design and Methodology. Rheumatology. 2019;58:kez063. 060.336. https://doi.org/10.1093/rheumatology/kez 063.060
71. Cid MC, Unizony S, Pupim L, Fang F, Pirrello J. Mavrilimumab (Anti GM-CSF Receptor Α Monoclonal Antibody) Reduces Risk of Flare and Increases Sustained Remission in a Phase 2 Trial of Patients with Giant Cell Arteritis. Ann Rheum Dis. 2021;80:31-32.
72. Cid MC, Unizony SH, Blockmans D, Brouwer E, Dagna L et al. Efficacy and safety of mavrilimumab in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2022;81(5):653-661. https://doi.org/10.1136/annrheumdis-2021-221865
73. Unizony S, Cid MC, Brouwer E, Dagna L, Dasgupta B et al. AB0370 Utility of CRP and ESR in the Diagnosis of Giant Cell Arteritis Relapse in a Phase 2 Trial of Mavrilimumab. Ann Rheum Dis. 2021;80:1211-1212.
74. Raffray L, Guillevin L. Rituximab treatment of ANCA-associated vasculitis. Expert Opin Biol Ther. 2020;20(8):899-910. doi: 10.1080/14712598.2020.1748597
75. Biologics in Refractory Vasculitis: A Trial of Biologic Therapy for Refractory Primary Non-ANCA Associated Vasculitis (BIOVAS). Clinical Trials.gov identifier: NCT05168475. Updated 29 May 2025. Accessed 9 July 2025. http://clinicaltrials.gov/study/NCT05168475
76. Bhatia A, Ell PJ, Edwards JC. Anti-CD20 monoclonal antibody (rituximab) as an adjunct in the treatment of giant cell arteritis. Ann Rheum Dis. 2005;64:1099e100.
77. Mayrbaeurl B, Hinterreiter M, Burgstaller S, Windpessl M, Thaler J. The first case ofa patient with neutropenia and giant-cell arteritis treated with rituximab. Clin Rheumatol. 2007;26:1597–8.
78. Ruch J, McMahon B, Ramsey G, Kwaan HC. Catastrophic multiple organ ischemia due to an anti-Pr cold agglutinin developing in a patient with mixed cryoglobulinemia after treatment with rituximab. Am J Hematol. 2009;84(2):120-2. doi: 10.1002/ajh.21330
79. Lu-Emerson C, Walker M, Huber BR, Ghodke B, Longstreth WT Jr et al. Lethal giant cell arteritis with multiple ischemic strokes despite aggressive immunosuppressive therapy. J Neurol Sci. 2010;295(1-2):120-4. doi: 10.1016/j.jns.2010.05. 008
80. Larivière D, Sacre K, Klein I, Hyafil F, Choudat L et al. Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study. Medicine (Baltimore). 2014;93(28):e265. doi: 10.1097/MD.0000000000 000265
81. Czihal M, Piller A, Schroettle A, Kuhlencordt P, Bernau C et al. Impact of cranial and axillary/subclavian artery involvement by color duplex sonography on response to treatment in giant cell arteritis. J Vasc Surg. 2015;61(5):1285-91. doi: 10.1016/j.jvs.2014.12.045
82. Pradhan D, Amin RM, Jones MW, Surti U, Parwani AV. Giant Cell Arteritis of the Female Genital Tract With Occult Temporal Arteritis and Marginal Zone Lymphoma Harboring Novel 20q Deletion: A Case Report and Literature Review. Int J Surg Pathol. 2016;24(1):78-84. doi: 10.1177/106689691560 5165
83. Ng WL, McManus J, Devlin JA, Fraser A. Unmasking the elusive giant: an unusual case presenting as third nerve palsy in a patient with scleroderma. BMJ Case Rep. 2016;2016:10.1136/bcr-2016-214633. doi: 10.1136/bcr-2016-214633
84. Hassane HH, Beg MM, Siva C, Velázquez C. Co-Presentation of Giant Cell Arteritis and Granulomatosis with Polyangiitis: A Case Report and Review of Literature. Am J Case Rep. 2018;19:651-655. doi: 10.12659/AJCR.909243
85. Mulhearn B, Cooper E, Knights S. Rituximab fails to treat giant cell arteritis in a patient with ACPA-positive rheumatoid arthritis. Rheumatol Adv Pract. 2021;5(1):rkab020. doi: 10.1093/rap/ rkab020
86. Shibata A, Kondo T, Kurasawa T, Chino K, Okada Y et al. A case of polyangiitis overlap syndrome of giant cell arteritis and granulomatosis with polyangiitis successfully treated with rituximab. Mod Rheumatol Case Rep. 2021;5(2):317-321. doi: 10.1080/24725625.2020.1780003
87. Coattrenec Y, Muller YD, Spoerl D, Lobrinus JA, Seebach JD. Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study. Rheumatol Int. 2021;41(12):2147-2156. doi: 10.1007/s00296-021-04993-2
88. Prünte MKR, Naumann A, Christ M, Naumann M, Bayas A. Giant cell arteritis with vertebral artery involvement-baseline characteristics and follow-up of a monocentric patient cohort. Front Neurol. 2023;14:1188073. doi: 10.3389/fneur.2023. 1188073
89. Roskoski R Jr.. Janus kinase (JAK) inhibitors in the treatment of neoplastic and inflammatory disorders. Pharmacol Res. 2022;183:106362. doi: 10.1016/j.phrs.2022.106362
90. Choy EH. Clinical significance of Janus Kinase inhibitor selectivity. Rheumatology (Oxford). 2019;58(6):953-962. doi: 10.1093/rheumatology/ key339
91. Nash P, Kerschbaumer A, Konzett V, Aletaha D, Dörner T et al. Expert consensus statement on the treatment of immune-mediated inflammatory diseases with Janus kinase inhibitors: 2024 update. Ann Rheum Dis. 2025;84(5):664-679. doi: 10.1016/j.ard.2025.01.032
92. Baccelli F, Gottardi F, Muratore E, Leardini D, Grasso AG et al. Ruxolitinib for the treatment of acute and chronic graft-versus-host disease in children: a systematic review and individual patient data meta-analysis. Bone Marrow Transplant. 2024;59(6):765-776. doi: 10.1038/s41409-024- 02252-z
93. Baricitinib in Relapsing Giant Cell Arteritis (GCA): A Phase II, Single-institution, Open-label Pilot Study. Clinical Trials.gov identifier: NCT03026504. Updated 5 March 2020. Accessed 10 July 2025. http://clinicaltrials.gov/study/NCT03026504
94. Koster MJ, Crowson CS, Giblon RE, Jaquith JM, Duarte-García A et al. Baricitinib for relapsing giant cell arteritis: a prospective open-label 52-week pilot study. Ann Rheum Dis. 2022;81(6):861-867. doi: 10.1136/annrheumdis-2021-221961
95. Régent A, Terrier B, Legendre P, Wartski M, Cohen P et al. Efficacy of baricitinib for refractory large-vessel vasculitis. Rheumatology (Oxford). 2021;60(11):e389-e391. doi: 10.1093/rheumatology/ keab541
96. Prigent K, Aouba A, Aide N, de Boysson H. JAK Inhibitor Effectiveness in Giant-Cell Arteritis With Large-Vessel Involvement Assessed by 18F-FDG PET-CT. Clin Nucl Med. 2022;47(3):234-235. doi: 10.1097/RLU.0000000000003913
97. Camellino D, Dejaco C, Martini F, Cosso R, Bianchi G. Baricitinib in polymyalgia rheumatica and giant cell arteritis: report of six cases. Reumatismo. 2025;77(1). doi: 10.4081/reumatismo.2024.1796
98. A Multicenter, Randomized, Double-Blind, Placebo- Controlled Study to Evaluate the Safety and Efficacy of Upadacitinib in Subjects With Giant Cell Arteritis: SELECT-GCA. Clinical Trials.gov identifier: NCT03725202. 19 March 2025. Accessed 10 July 2025. http://clinicaltrials.gov/study/NCT03725202
99. Blockmans D, Penn SK, Setty AR, Schmidt WA, Rubbert-Roth A et al. A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis. N Engl J Med. 2025;392(20):2013-2024. doi: 10.1056/NEJMoa2 413449
100. Sanada A, Abe N, Bohgaki M, Kasahara H. Therapeutic effectiveness of upadacitinib combined with glucocorticoid on remission induction and maintenance in giant cell arteritis, Rheumatology. 2022;61(9):e274–e276, https://doi.org/10.1093/rheumatology/keac203
101. Matza MA, Dagincourt N, Mohan SV, Pavlov A, Han J et al. Outcomes during and after long-term tocilizumab treatment in patients with giant cell arteritis. RMD Open. 2023;9(2):e002923. doi: 10.1136/rmdopen-2022-002923
102. Eriksson P, Skoglund O, Hemgren C, Sjöwall C. Clinical experience and safety of Janus kinase inhibitors in giant cell arteritis: a retrospective case series from Sweden. Front Immunol. 2023;14:1187584. doi: 10.3389/fimmu.2023. 1187584
103. Loricera J, Tofade T, Prieto-Peña D, Romero-Yuste S, de Miguel E et al. Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature. Arthritis Res Ther. 2024;26(1):116. doi: 10.1186/s13075-024-03314-9
104. Herlihy N, Curto-Garcia N, O'Sullivan J, Radia D, McLornan D et al. Successful treatment of chronic neutrophilic leukaemia and associated giant cell arteritis with the combination of ruxolitinib and azacytidine. Supplement Article. Br J Haematol. 2019;185(S1):3–202. doi: 10.1111/bjh.15854
105. Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020;79(1):19-30. doi: 10.1136/annrheumdis-2019-215672
106. Maz M, Chung SA, Abril A, Langford CA, Gorelik M et al. 2021 American College of Rheumatology/ Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365. doi: 10.1002/art.41774
doi: 10.1016/j.semarthrit.2025.152686
2. Hoffman GS. Giant Cell Arteritis. Ann Intern Med. 2016;165(9):ITC65-ITC80.
doi: 10.7326/AITC201611010
3. Cho HJ, Bloomberg J, Nichols J. Giant cell arteritis. Dis Mon. 2017;63(3):88-91.
doi: 10.1016/j.disamonth.2016.10.006
4. Watelet B, Samson M, de Boysson H, Bienvenu B. Treatment of giant-cell arteritis, a literature review. Mod Rheumatol. 2017;27(5):747-754. doi: 10.1080/14397595.2016.1266070
5. Stone JH, McDowell PJ, Jayne DRW, Merkel PA, Robson J et al. The glucocorticoid toxicity index: Measuring change in glucocorticoid toxicity over time. Semin Arthritis Rheum. 2022 ;55:152010. doi: 10.1016/j.semarthrit.2022.152010
6. Wilson JC, Sarsour K, Collinson N, Tuckwell K, Musselman D et al. Incidence of outcomes potentially associated with corticosteroid therapy in patients with giant cell arteritis. Semin Arthritis Rheum. 2017;46(5):650-656. doi: 10.1016/j.semarthrit.2016.10.001
7. Wilson JC, Sarsour K, Collinson N, Tuckwell K, Musselman D et al. Serious adverse effects associated with glucocorticoid therapy in patients with giant cell arteritis (GCA): A nested case-control analysis. Semin Arthritis Rheum. 2017;46(6):819-827. doi: 10.1016/j.semarthrit.2016.11.006
8. Buttgereit F, Dejaco C, Matteson EL, Dasgupta B. Polymyalgia Rheumatica and Giant Cell Arteritis: A Systematic Review. JAMA. 2016;315(22):2442-58. doi: 10.1001/jama.2016.5444
9. Jover JA, Herna´ndez-Garcı´a C, Morado IC, Vargas E, Bañares A et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone. a randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2001;134:106–14.
10. Koster MJ, Yeruva K, Crowson CS, Muratore F, Labarca C et al. Efficacy of methotrexate in real-world management of giant cell arteritis: a case-control study. J Rheumatol. 2019;46: 501–8.
11. Leon L, Rodriguez-Rodriguez L, Morado I, Rosales Z, Vadillo C et al. Treatment with methotrexate and risk of relapses in patients with giant cell arteritis in clinical practice. Clin Exp Rheumatol. 2018;36:121–8.
12. Kramariˇ c J, Rotar ˇ Z, Tomˇ siˇ c M, Hoˇ cevar A. Performance of leflunomide as a steroid-sparing agent in giant cell arteritis: a single-center, open-label study. Front Med. 2022;9. https://doi.org/10.3389/fmed.2022.1069013
13. Hoˇ cevar A, Jeˇ se R, Rotar ˇ Z, Tomˇ siˇ c M. Does leflunomide have a role in giant cell arteritis? An open-label study. Clin Rheumatol. 2019;38(2):291–6. http://doi.org/10.1007/s10067-018-4232-x
14. Hoffman GS, Cid MC, Rendt-Zagar KE, Merkel PA, Weyand CM et al. Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med. 2007;146:621–30.
15. Seror R, Baron G, Hachulla E, Debandt M, Larroche M et al. Adalimumab for steroid sparing in patients with giant-cell arteritis: results of a multicentre randomised controlled trial. Ann Rheum Dis. 2014;73:2074–81.
16. Martı´nez-Taboada VM, Rodrı´guez-Valverde V, Carreno~ L, López-Longo J, Figueroa M, et al. A double-blind placebo controlled trial of etanercept in patients with giant cell arteritis and corticosteroid side effects. Ann Rheum Dis. 2008;67:625–30.
17. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377:317–28.
18. Villiger PM, Adler S, Kuchen S, Wermelinger F, Dan D et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebocontrolled trial. Lancet. 2016;387:1921–7.
19. European Medicines Agency. RoActemra tocilizumab new indication. Available from, https://www.ema.europa.eu/en/documents/smop/chmp-post-authorisation-summary-positive-opinion-roactemra-ii-66_en.pdf; 2023
20. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017;377(4):317–28. https://doi.org/10.1056/NEJMoa1613849
21. Samson M, Dasgupta B, Sammel AM, Salvarani C, Pagnoux C et al. Targeting interleukin-6 pathways in giant cell arteritis management: A narrative review of evidence. Autoimmun Rev. 2025 Jan;24(2):103716. doi: 10.1016/j.autrev.2024.103716
22. Schmidt WA, Dasgupta B, Luqmani R, Unizony SH, Blockmans D, et al. A multicentre, randomised, doubleblind, placebo-controlled, parallel-group study to evaluate the efficacy and safety of Sirukumab in the treatment of Giant cell arteritis. Rheumatol Ther. 2020;7(4): 793–810. https://doi.org/10.1007/s40744-020-00227-2
23. A Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Sarilumab in Patients With Giant Cell Arteritis. ClinicalTrials.gov identifier: NCT03600805. Updated 15 March 2022. Accessed 24 June 2025. https://clinicaltrials.gov/study/NCT03600805
24. Pombo-Suarez M, Gomez-Reino JJ. Abatacept for the treatment of rheumatoid arthritis. Expert Rev Clin Immunol. 2019;15(4):319-326. doi: 10.1080/1744666X.2019.1579642
25. Abatacept for Treating Adults With Giant Cell Arteritis and Takayasu's Arteritis. ClinicalTrials.gov identifier: NCT00556439. Updated January 25, 2018. Accessed 24 June 2025. https://clinicaltrials.gov/study/NCT00556439
26. A Study to Evaluate Efficacy and Safety of Subcutaneous Abatacept With Steroid Treatment Compared to Steroid Treatment Alone in Adults With Giant Cell Arteritis (GCA). ClinicalTrials.gov identifier: NCT03192969. Updated July 10, 2017. Accessed 26 June 2025. https://clinicaltrials.gov/study/NCT03192969
27. Abatacept for the Treatment of Giant Cell Arteritis. ClinicalTrials.gov identifier: NCT04474847. Updated 10 February, 2025. Accessed 27 June 2025. https://clinicaltrials.gov/study/NCT04474847
28. Langford CA, Cuthbertson D, Ytterberg SR, Khalidi N, Monach PA,et al. A Randomized, Double-Blind Trial of Abatacept (CTLA-4Ig) for the Treatment of Giant Cell Arteritis. Arthritis Rheumatol. 2017;69(4): 837-845. doi: 10.1002/art.40044
29. Gérard AL, Simon-Tillaux N, Yordanov Y, Cacoub P, Tubach F et al. Efficacy and safety of steroid-sparing treatments in giant cell arteritis according to the glucocorticoids tapering regimen: A systematic review and meta-analysis. Eur J Intern Med. 2021;88:96-103. doi: 10.1016/j.ejim.2021. 03.04
30. Rossi D, Cecchi I, Sciascia S, Naretto C, Alpa M et al. An agent-to-agent real life comparison study of tocilizumab versus abatacept in giant cell arteritis. Clin Exp Rheumatol. 2021;39 Suppl 129(2):125-128. doi: 10.55563/clinexprheumatol/l0hd9v
31. Caton MT Jr, Mark IT, Narsinh KH, Baker A, Cooke DL et al. Endovascular Therapy for Intracranial Giant Cell Arteritis : Systematic Review, Technical Considerations and the Effect of Intra-arterial Calcium Channel Blockers. Clin Neuroradiol. 2022;32(4):1045-1056. doi: 10.1007/s00062-022-01171-0
32. Arnold DD, Yalamanoglu A, Boyman O. Systematic Review of Safety and Efficacy of IL-1-Targeted Biologics in Treating Immune-Mediated Disorders. Front Immunol. 2022;13:888392. doi: 10.3389/fimmu.2022.888392
33. Giant Cell Arteritis and Anakinra Trial (GiAnT). ClinicalTrials.gov identifier: NCT02902731. Updated 21 February 2021. Accessed 28 June 2025. https://clinicaltrials.gov/study/NCT02902731
34. De Boysson H, Ly KH, Geffray L, Quemeneur T, Liozon E et al. Four months of treatment with anakinra combined with glucocorticoids for giant cell arteritis: a multicenter, randomized, double-blind, placebo-controlled trial. Arthritis Res Ther. 2025;27(1):122. doi: 10.1186/s13075-025-03493-z
35. Deshayes S, Ly KH, Rieu V, Maigné G, Martin Silva N et al. Steroid-sparing effect of anakinra in giant-cell arteritis: a case series with clinical, biological and iconographic long-term assessments. Rheumatology (Oxford). 2021;61(1):400-406. doi: 10.1093/rheumatology/keab280
36. Ly KH, Stirnemann J, Liozon E, Michel M, Fain O et al. Interleukin-1 blockade in refractory giant cell arteritis. Joint Bone Spine. 2014;81(1):76-8. doi: 10.1016/j.jbspin.2013.06.004
37. Gallou S, Agard C, Dumont A, Deshayes S, Boutemy J et al. Evolution and outcomes of aortic dilations in giant cell arteritis. Eur J Intern Med. 2024;129:71-77. doi: 10.1016/j.ejim.2024.03.038
38. Dumont A, Parienti JJ, Delmas C, Boutemy J, Maigné G et al. Factors Associated with Relapse and Dependence on Glucocorticoids in Giant Cell Arteritis. J Rheumatol. 2020;47(1):108-116. doi: 10.3899/jrheum.181127
39. Rossi-Semerano L, Fautrel B, Wendling D, Hachulla E, Galeotti C et al. Tolerance and efficacy of off-label anti-interleukin-1 treatments in France: a nationwide survey. Orphanet J Rare Dis. 2015;10:19. doi: 10.1186/s13023-015-0228-7
40. De Boysson H, Liozon E, Espitia O, Daumas A, Vautier M et al. Different patterns and specific outcomes of large-vessel involvements in giant cell arteritis. J Autoimmun. 2019;103:102283. doi: 10.1016/j. jaut.2019. 05.011
41. A randomised, double-blind, placebo-controlled proof-of concept study of the efficacy and safety of gevokizumab in the treatment of patients with giant cell arteritis. EudraCT number: 2013-002778-38 Updated 4 December 2016. Accessed 28 June 2025.
42. Andel PM, Diamantopoulos AP, Myklebust G, Haugeberg G. Vasculitis distribution and clinical characteristics in giant cell arteritis: a retrospective study using the new 2022 ACR/EULAR classification criteria. Front Med (Lausanne). 2023;10:1286601. doi: 10.3389/fmed.2023.1286
43. Roberts J, O'Rielly DD, Rahman P. A review of ustekinumab in the treatment of psoriatic arthritis. Immunotherapy. 2018;10(5):361-372. doi: 10.2217/imt-2017-0149
44. Ustekinumab for the Treatment of Giant Cell Arteritis (UGCA) ClinicalTrials.gov identifier: NCT02955147. Updated 10 June 2020. Accessed 7 July 2025. https://clinicaltrials.gov/study/NCT02955147
45. Matza MA, Fernandes AD, Stone JH, Unizony SH. Ustekinumab for the treatment of Giant cell arteritis. Arthritis Care Res. 2021;73:893–7. https://doi.org/10.1002/ acr.24200
46. Ustekinumab for the Treatment of Relapse of Refractory Giant Cell Arteritis (ULTRA). ClinicalTrials.gov identifier: NCT03711448. Updated 3 October 2024. Accessed 7 July 2025. https://clinicaltrials.gov/study/NCT03711448
47. Conway R, O’Neill L, O’Flynn E, Gallagher P, McCarthy GM et al. Ustekinumab for the treatment of refractory giant cell arteritis. Ann Rheum Dis. 2016;75:1578–9
48. Conway R, O’Neill L, Gallagher P, McCarthy GM, Murphy CC et al. Ustekinumab for refractory giant cell arteritis: A prospective 52-week trial. Semin Arthritis Rheum. 2018;48:523–8.
49. Samson M, Ghesquière T, Berthier S, Bonnotte B. Ustekinumab inhibits Th1 and Th17 polarisation in a patient with giant cell arteritis. Ann Rheum Dis. 2018;77:e6. doi: 10.1136/annrheumdis-2017-211622
50. Abdalla A, Ali I, Murphy D, Molloy E. Connecting the dots: a story of unknown fever, acute coronary syndrome and pan-aortitis-an occult relapse of a large vessel vasculitis. BMJ Case Rep. 2019;12(5):e230424. doi: 10.1136/bcr-2019
51. Maillet F, Nguyen Y, Espitia O, Perard L, Salvarani C et al. Association between large vessel vasculitis and inflammatory bowel disease: a case-control study. Rheumatology(Oxford). 2025;64(6):3724-3732. doi: 10.1093/rheumatology/keaf030
52. Sandler RD, Smith R, Kitsanta P, Hughes M. Paradoxical New Diagnosis of Giant Cell Arteritis While Receiving Ustekinumab. J Clin Rheumatol. 2020;26(7):e215-e216. doi: 10.1097/RHU.0000000000001084
53. Samson M, Bonnotte B. Ustekinumab fo the Treatment of Giant Cell Arteritis: Comment on the Articls by Matza et al. Arthritis Care Res. 2021;73:1058-1059.
54. Conway R, Molloy ES. Ustekinumab in Giant Cell Arteritis: Comment on the Article by Matza et al. Arthritis Care Res. 2021;73:1056–1057.
55. Fauny M, Moulin D, D'Amico F, Netter P, Petitpain N et al. Paradoxical gastrointestinal effects of interleukin-17 blockers. Ann Rheum Dis. 2020;79(9):1132-1138. doi: 10.1136/annrheumdis-2020-217927
56. Study to Evaluate the Pharmacokinetics, Safety and Tolerability of Intravenous Secukinumab in Patients With GCA or PMR ClinicalTrials.gov identifier: NCT06130540. Updated 3 June 2025. Accessed 8 July 2025. https://clinicaltrials.gov/study/NCT06130540
57. A Placebo-controlled Phase 2 Trial to Investigate the Safety and Efficacy of Secukinumab in Giant Cell Arteritis (TitAIN). ClinicalTrials.gov identifier: NCT03765788. Updated 16 August 2023. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT03765788
58. Venhoff N, Schmidt WA, Lamprecht P, Tony HP, App C et al. Efficacy and safety of secukinumab in patients with giant cell arteritis: study protocol for a randomized, parallel group, double-blind, placebo-controlled phase II trial. Trials.2021;22 (1):543. doi: 10.1186/s13063-021-05520-1
59. Venhoff N, Schmidt WA, Bergner R, Rech J, Unger L et al. Safety and efficacy of secukinumab in patients with giant cell arteritis (TitAIN): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Rheumatol. 2023;5(6):e341–e50. doi: 10.1016/S2665- 9913(23)00101-7
60. Phase III Study of Efficacy and Safety of Secukinumab Versus Placebo, in Combination With Glucocorticoid Taper Regimen, in Patients With Giant Cell Arteritis (GCA) (GCAptAIN). ClinicalTrials.gov identifier: NCT04930094. Updated 6 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT04930094
61. Efficacy and Safety of Secukinumab in Patients With New Onset of Giant Cell Arteritis Who Are in Clinical Remission (GigAINt). ClinicalTrials.gov identifier: NCT05380453. Updated 27 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT05380453
62. Tomelleri A, Bond M, Marvisi C, Campochiaro C, Farina N et al. Secukinumab is effective and safe for patients with giant cell arteritis after tocilizumab failure. Rheumatology (Oxford). 2025:keaf250. doi: 10.1093/rheumatology/keaf250
63. Rotar ˇZ, Tomˇsiˇc M, Hoˇcevar A. Secukinumab for the maintenance of glucocorticoid-free remission in a patient with giant cell arteritis and psoriatic arthritis. Rheumatology. 2018;57:934–6. https://doi.org/10.1093/rheumatology/kex507
64. Sammut L, Litwic A, Smith R, Bartram S. 002 Biopsy proven giant cell arteritis in a patient with psoriatic arthritis on TNF-alpha inhibitor treated with high dose prednisolone and a switch to secukinumab (anti IL-17). Rheumatology. 2018;57(suppl_3):key075. 226.
65. Tomelleri A, Rinaldi E, Campochiaro C, Picchio M, Dagna L. Successful use of ixekizumab for glucocorticoid-free remission maintenance in giant cell arteritis. Rheumatology (Oxford). 2023;62(2):e24-e26. doi: 10.1093/rheumatology/ keac416
66. Ghoreschi K, Balato A, Enerbäck C, Sabat R. Therapeutics targeting the IL-23 and IL-17 pathway in psoriasis. Lancet. 2021;397(10275):754-766. doi: 10.1016/S0140-6736(21)00184-7
67. A Study to Evaluate Guselkumab for the Treatment of Participants With New-onset or Relapsing Giant Cell Arteritis (THEIA). Clinical Trials.gov identifier: NCT04633447. Updated 26 June 2025. Accessed 8 July 2025. http://clinicaltrials.gov/study/NCT04633447
68. Crotti C, Biggioggero M, Becciolini A, Agape E, Favalli EG. Mavrilimumab: a unique insight and update on the current status in the treatment of rheumatoid arthritis. Expert Opin Investig Drugs. 2019;28(7):573-581. doi: 10.1080/13543784. 2019.1631795
69. A Phase 2, Randomized, Double-blind Placebo- controlled Study to Test the Efficacy and Safety of KPL-301 in Giant Cell Arteritis. Clinical Trials.gov identifier: NCT03827018. Updated 17 October 2023. Accessed 9 July 2025. http://clinicaltrials.gov/study/NCT0382701
70. Pupim L, Unizony S, Cid M, Pilipski L, Gandhi R et al. A Phase 2, Randomized, Double-Blind Placebo-Controlled Study to Test The Efficacy and Safety of Mavrilimumab in Giant Cell Arteritis: Study Design and Methodology. Rheumatology. 2019;58:kez063. 060.336. https://doi.org/10.1093/rheumatology/kez 063.060
71. Cid MC, Unizony S, Pupim L, Fang F, Pirrello J. Mavrilimumab (Anti GM-CSF Receptor Α Monoclonal Antibody) Reduces Risk of Flare and Increases Sustained Remission in a Phase 2 Trial of Patients with Giant Cell Arteritis. Ann Rheum Dis. 2021;80:31-32.
72. Cid MC, Unizony SH, Blockmans D, Brouwer E, Dagna L et al. Efficacy and safety of mavrilimumab in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Ann Rheum Dis. 2022;81(5):653-661. https://doi.org/10.1136/annrheumdis-2021-221865
73. Unizony S, Cid MC, Brouwer E, Dagna L, Dasgupta B et al. AB0370 Utility of CRP and ESR in the Diagnosis of Giant Cell Arteritis Relapse in a Phase 2 Trial of Mavrilimumab. Ann Rheum Dis. 2021;80:1211-1212.
74. Raffray L, Guillevin L. Rituximab treatment of ANCA-associated vasculitis. Expert Opin Biol Ther. 2020;20(8):899-910. doi: 10.1080/14712598.2020.1748597
75. Biologics in Refractory Vasculitis: A Trial of Biologic Therapy for Refractory Primary Non-ANCA Associated Vasculitis (BIOVAS). Clinical Trials.gov identifier: NCT05168475. Updated 29 May 2025. Accessed 9 July 2025. http://clinicaltrials.gov/study/NCT05168475
76. Bhatia A, Ell PJ, Edwards JC. Anti-CD20 monoclonal antibody (rituximab) as an adjunct in the treatment of giant cell arteritis. Ann Rheum Dis. 2005;64:1099e100.
77. Mayrbaeurl B, Hinterreiter M, Burgstaller S, Windpessl M, Thaler J. The first case ofa patient with neutropenia and giant-cell arteritis treated with rituximab. Clin Rheumatol. 2007;26:1597–8.
78. Ruch J, McMahon B, Ramsey G, Kwaan HC. Catastrophic multiple organ ischemia due to an anti-Pr cold agglutinin developing in a patient with mixed cryoglobulinemia after treatment with rituximab. Am J Hematol. 2009;84(2):120-2. doi: 10.1002/ajh.21330
79. Lu-Emerson C, Walker M, Huber BR, Ghodke B, Longstreth WT Jr et al. Lethal giant cell arteritis with multiple ischemic strokes despite aggressive immunosuppressive therapy. J Neurol Sci. 2010;295(1-2):120-4. doi: 10.1016/j.jns.2010.05. 008
80. Larivière D, Sacre K, Klein I, Hyafil F, Choudat L et al. Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study. Medicine (Baltimore). 2014;93(28):e265. doi: 10.1097/MD.0000000000 000265
81. Czihal M, Piller A, Schroettle A, Kuhlencordt P, Bernau C et al. Impact of cranial and axillary/subclavian artery involvement by color duplex sonography on response to treatment in giant cell arteritis. J Vasc Surg. 2015;61(5):1285-91. doi: 10.1016/j.jvs.2014.12.045
82. Pradhan D, Amin RM, Jones MW, Surti U, Parwani AV. Giant Cell Arteritis of the Female Genital Tract With Occult Temporal Arteritis and Marginal Zone Lymphoma Harboring Novel 20q Deletion: A Case Report and Literature Review. Int J Surg Pathol. 2016;24(1):78-84. doi: 10.1177/106689691560 5165
83. Ng WL, McManus J, Devlin JA, Fraser A. Unmasking the elusive giant: an unusual case presenting as third nerve palsy in a patient with scleroderma. BMJ Case Rep. 2016;2016:10.1136/bcr-2016-214633. doi: 10.1136/bcr-2016-214633
84. Hassane HH, Beg MM, Siva C, Velázquez C. Co-Presentation of Giant Cell Arteritis and Granulomatosis with Polyangiitis: A Case Report and Review of Literature. Am J Case Rep. 2018;19:651-655. doi: 10.12659/AJCR.909243
85. Mulhearn B, Cooper E, Knights S. Rituximab fails to treat giant cell arteritis in a patient with ACPA-positive rheumatoid arthritis. Rheumatol Adv Pract. 2021;5(1):rkab020. doi: 10.1093/rap/ rkab020
86. Shibata A, Kondo T, Kurasawa T, Chino K, Okada Y et al. A case of polyangiitis overlap syndrome of giant cell arteritis and granulomatosis with polyangiitis successfully treated with rituximab. Mod Rheumatol Case Rep. 2021;5(2):317-321. doi: 10.1080/24725625.2020.1780003
87. Coattrenec Y, Muller YD, Spoerl D, Lobrinus JA, Seebach JD. Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study. Rheumatol Int. 2021;41(12):2147-2156. doi: 10.1007/s00296-021-04993-2
88. Prünte MKR, Naumann A, Christ M, Naumann M, Bayas A. Giant cell arteritis with vertebral artery involvement-baseline characteristics and follow-up of a monocentric patient cohort. Front Neurol. 2023;14:1188073. doi: 10.3389/fneur.2023. 1188073
89. Roskoski R Jr.. Janus kinase (JAK) inhibitors in the treatment of neoplastic and inflammatory disorders. Pharmacol Res. 2022;183:106362. doi: 10.1016/j.phrs.2022.106362
90. Choy EH. Clinical significance of Janus Kinase inhibitor selectivity. Rheumatology (Oxford). 2019;58(6):953-962. doi: 10.1093/rheumatology/ key339
91. Nash P, Kerschbaumer A, Konzett V, Aletaha D, Dörner T et al. Expert consensus statement on the treatment of immune-mediated inflammatory diseases with Janus kinase inhibitors: 2024 update. Ann Rheum Dis. 2025;84(5):664-679. doi: 10.1016/j.ard.2025.01.032
92. Baccelli F, Gottardi F, Muratore E, Leardini D, Grasso AG et al. Ruxolitinib for the treatment of acute and chronic graft-versus-host disease in children: a systematic review and individual patient data meta-analysis. Bone Marrow Transplant. 2024;59(6):765-776. doi: 10.1038/s41409-024- 02252-z
93. Baricitinib in Relapsing Giant Cell Arteritis (GCA): A Phase II, Single-institution, Open-label Pilot Study. Clinical Trials.gov identifier: NCT03026504. Updated 5 March 2020. Accessed 10 July 2025. http://clinicaltrials.gov/study/NCT03026504
94. Koster MJ, Crowson CS, Giblon RE, Jaquith JM, Duarte-García A et al. Baricitinib for relapsing giant cell arteritis: a prospective open-label 52-week pilot study. Ann Rheum Dis. 2022;81(6):861-867. doi: 10.1136/annrheumdis-2021-221961
95. Régent A, Terrier B, Legendre P, Wartski M, Cohen P et al. Efficacy of baricitinib for refractory large-vessel vasculitis. Rheumatology (Oxford). 2021;60(11):e389-e391. doi: 10.1093/rheumatology/ keab541
96. Prigent K, Aouba A, Aide N, de Boysson H. JAK Inhibitor Effectiveness in Giant-Cell Arteritis With Large-Vessel Involvement Assessed by 18F-FDG PET-CT. Clin Nucl Med. 2022;47(3):234-235. doi: 10.1097/RLU.0000000000003913
97. Camellino D, Dejaco C, Martini F, Cosso R, Bianchi G. Baricitinib in polymyalgia rheumatica and giant cell arteritis: report of six cases. Reumatismo. 2025;77(1). doi: 10.4081/reumatismo.2024.1796
98. A Multicenter, Randomized, Double-Blind, Placebo- Controlled Study to Evaluate the Safety and Efficacy of Upadacitinib in Subjects With Giant Cell Arteritis: SELECT-GCA. Clinical Trials.gov identifier: NCT03725202. 19 March 2025. Accessed 10 July 2025. http://clinicaltrials.gov/study/NCT03725202
99. Blockmans D, Penn SK, Setty AR, Schmidt WA, Rubbert-Roth A et al. A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis. N Engl J Med. 2025;392(20):2013-2024. doi: 10.1056/NEJMoa2 413449
100. Sanada A, Abe N, Bohgaki M, Kasahara H. Therapeutic effectiveness of upadacitinib combined with glucocorticoid on remission induction and maintenance in giant cell arteritis, Rheumatology. 2022;61(9):e274–e276, https://doi.org/10.1093/rheumatology/keac203
101. Matza MA, Dagincourt N, Mohan SV, Pavlov A, Han J et al. Outcomes during and after long-term tocilizumab treatment in patients with giant cell arteritis. RMD Open. 2023;9(2):e002923. doi: 10.1136/rmdopen-2022-002923
102. Eriksson P, Skoglund O, Hemgren C, Sjöwall C. Clinical experience and safety of Janus kinase inhibitors in giant cell arteritis: a retrospective case series from Sweden. Front Immunol. 2023;14:1187584. doi: 10.3389/fimmu.2023. 1187584
103. Loricera J, Tofade T, Prieto-Peña D, Romero-Yuste S, de Miguel E et al. Effectiveness of janus kinase inhibitors in relapsing giant cell arteritis in real-world clinical practice and review of the literature. Arthritis Res Ther. 2024;26(1):116. doi: 10.1186/s13075-024-03314-9
104. Herlihy N, Curto-Garcia N, O'Sullivan J, Radia D, McLornan D et al. Successful treatment of chronic neutrophilic leukaemia and associated giant cell arteritis with the combination of ruxolitinib and azacytidine. Supplement Article. Br J Haematol. 2019;185(S1):3–202. doi: 10.1111/bjh.15854
105. Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020;79(1):19-30. doi: 10.1136/annrheumdis-2019-215672
106. Maz M, Chung SA, Abril A, Langford CA, Gorelik M et al. 2021 American College of Rheumatology/ Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol. 2021;73(8):1349-1365. doi: 10.1002/art.41774