The treatment of GCA has been transformed recently by demonstration of the efficacy of IL-6 receptor (IL-6R) blockade for remission maintenance. The GiACTA trial—the first trial in any disease to employ a blinded, variable-dose glucocorticoid-tapering regimen —showed that tocilizumab is not only highly effective in maintaining disease remissions induced by the combination of tocilizumab and prednisone, but also that IL-6R blockade has a pronounced steroid-sparing effect for patients . Perhaps the most astonishing finding from this trial was just how poorly patients treated with glucocorticoids alone fared. In the best-case scenario—that which does not take into account serum CRP levels as a possible indicator of active disease—only 33% of the patients allocated to treatment with 1 full year of prednisone achieved and maintained disease remission. Tocilizumab is now increasingly available to GCA patients all over the world, marking the first unequivocal treatment advance in this disease in nearly 70 years. The decades-old standard of care of prednisone-only treatment badly needed an update, and it has gotten it. As striking and important as this treatment breakthrough is, however, the disease will continue to pose diagnostic and management challenges for years to come. GCA is characterized by protean clinical features that sometimes present in a classic, textbook fashion. Yet equally, if not more often it presents with symptoms and signs that are frighteningly subtle, defying diagnosis for weeks, months or longer. I have therefore assembled a group of outstanding GCA investigators to approach this disease from a number of angles. I am grateful to all of the contributors for their outstanding work. Banz and Stone  joined me in writing a collaboration between a pathologist and a clinician, providing pearls and pitfalls in the clinicopathological diagnosis of GCA. Buttgereit et al.  take a deep dive into the complications of glucocorticoids in GCA and how to do one’s best to avoid them. Schmidt , peerless in the realm of ultrasonographic evaluation in GCA, describes the current state of the art of this technology in the diagnosis and management of this disease. Koster et al.  penned a thorough review of large-vessel GCA that draws on the finest evidence available. Schett  considers the broad physiological implications of excess IL-6 states—and the implications of inhibiting the actions of this cytokine. Terrades-Garcia and Cid  drew upon their years of experience in studying GCA at the tissue level for an insightful and up-to-date description of disease pathophysiology. And finally, Vodopivec and Rizzo , my neuro-ophthalmology colleagues in Boston, wrote a detailed review of the most feared effects of GCA from the standpoint of patients, namely ocular complications. I anticipate that for the first time in decades, advances in treatment may actually outstrip growth in our understanding of the clinical, radiological and pathophysiological aspects of GCA. In fact, treatment advances may drive progress in these other areas. An unintended consequence of the new era of therapy in which we find ourselves will likely be a surge in efforts and opportunities to understand better how to diagnose and manage GCA and how to develop therapies that are even more effective than IL-6R blockade. These articles summarize the current states of knowledge of many of the most important aspects of GCA and will help point the way to new knowledge, advances in care and treatment breakthroughs. Acknowledgement Editorial assistance was provided by ApotheCom and funded by F. Hoffmann-La Roche Ltd. Supplement: This supplement was supported by F. Hoffmann-La Roche Ltd. Funding: No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article. Disclosure statement: J.H.S. has received research grants and consulting for Roche, Genentech and Xencor. References 1 Collinson N, Tuckwell K, Habeck F et al. Development and implementation of a double-blind corticosteroid-tapering regimen for a clinical trial. Int J Rheumatol 2015; 2015: 589841. Google Scholar PubMed 2 Stone JH, Tuckwell K, Dimonaco S et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med 2017; 377: 317– 28. Google Scholar CrossRef Search ADS PubMed 3 Banz Y, Stone JH. Why do temporal arteries go wrong? Principles and pearls from a clinician and a pathologist. Rheumatology 2018; 57(suppl 2): ii3– 10. 4 Buttgereit F, Matteson EL, Dejaco C, Dasgupta B. Prevention of glucocorticoid morbidity in giant cell arteritis. Rheumatology 2018; 57(suppl 2): ii11– 21. 5 Schmidt WA. Ultrasound in the diagnosis and management of giant cell arteritis. Rheumatology 2018; 57(suppl 2): ii22– 31. 6 Koster MJ, Matteson EL, Warrington KJ. Large-vessel giant cell arteritis: diagnosis, monitoring and management. Rheumatology 2018; 57(suppl 2): ii32– 42. 7 Schett G. Physiological effects of modulating the interleukin 6 axis. Rheumatology 2018; 57(suppl 2): ii43– 50. 8 Terrades-Garcia N, Cid MC. Pathogenesis of giant-cell arteritis: how targeted therapies are influencing our understanding of the mechanisms involved. Rheumatology 2018; 57(suppl 2): ii51– 62. 9 Vodopivec I, Rizzo IIIFJ. Ophthalmic manifestations of giant cell arteritis. Rheumatology 2018; 57(suppl 2): ii63– 71. © The Author(s) 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. 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Rheumatology – Oxford University Press
Published: Feb 1, 2018
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