Recent Advances in Giant Cell Arteritis
Purpose of Review
Giant cell arteritis (GCA) is the most common systemic vasculitis. GCA is categorized as a granulomatous vasculitis of large and medium size vessels. Majority of the symptoms and signs of GCA result from involvement of the aorta and its branches intra- and extracranial. Temporal artery biopsy continues to be the cardinal diagnostic procedure despite new imaging modalities for diagnosing GCA with cranial involvement. Great advances in awareness have led to improvement in preventing irreversible vision loss due to early diagnosis.
The cause of GCA has not been elucidated but major progress has been made in the knowledge of its pathogenesis leading to new therapeutic targets, particularly inhibition of interleukin 6. IL 6 plays a key role in the regulation of TH17/Tregs imbalance in GCA and appears to correlate with clinical disease activity in GCA. All of this has led to the first FDA (food and drug administration) approved treatment for GCA, Tocilizumab. Abatacept and Ustekinumab are promising targets for therapy in LVV but still need further research.
This paper is a review of the recent progress in the understanding of GCA pathogenesis, diagnosis, treatment, and prognosis.
KeywordsReview of epidemiology Pathophysiology Diagnosis Treatment and prognosis for GCA
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 10.Li Y, Liu Y, Strickland FM, Richardson B. Age-dependent decreases in DNA methyl-transferase levels and low transmethylation micronutrient levels synergize to promote overexpression of genes implicated in autoimmunity and acute coronary syndromes. Exp Gerontol. 2010;45:312–22.CrossRefPubMedGoogle Scholar
- 23.• Miyabe C, Miyabe Y, et al. An expanded population of pathogenic regulatory T cells in giant cell arteritis is abrogated by IL6 blockade therapy. Ann Rheum Dis. 2016. This article provides important information about the role of IL 6 in the imbalance of TH17/TH regulatory cells in the pathophysiology of GCA. Google Scholar
- 26.Corbera-Bellalta M, Planas-Rigol E. Blocking interferon gamma reduces expression of chemokines CXCL9, CXCL10 and CXCL11 and decreases macrophage infiltration in ex vivo cultured arteries from patients with giant cell arteritis. Ann Rheum. 2015.Google Scholar
- 31.Nuenninghoff DM, Hunder GG, Christianson TJ, McClelland RL, Matteson EL. Incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis: a population-based study over 50 years. Arthritis Rheum. 2003;4(8):3522–31.CrossRefGoogle Scholar
- 34.Cid MC, Font C, Oristrell J, de la Sierra A, Coll-Vinent B, López-Soto A, et al. Association between strong inflammatory response and low risk of developing visual loss and other cranial ischemic complications in giant cell (temporal) arteritis. Arthritis Rheum. 1998;41(1):26–32.CrossRefPubMedGoogle Scholar
- 58.Perez-Lopez J, Solans Laque R, et al. Colour-duplex ultrasonography of the temporal and ophthalmic arteries in the diagnosis and follow up of giant cell arteritis. Clin Exp Rheumatol. 2009;27:311–7.Google Scholar
- 59.Ghinoi A, Pipitone N, Nicolini A, Boiardi L, Silingardi M, Germano G, et al. Large-vessel involvement in recent onset giant cell arteritis: a case-control colour Doppler sonography study. Rheumatology (Oxford). 2012;36(9):730–4.Google Scholar
- 61.Mackie SL, Hensor EM, et al. Should I send my patient with previous giant cell arteritis for imaging of the thoracic aorta? A systematic literature review and meta-analysis. Ann Rheum Dis. Published Online First 22 Dec 2012.Google Scholar
- 64.Grayson PC, Alehashemi S, Bagheri AA, Civelek AC, et al. 18 F-Fluorodeoxyglucose-Positron Emission Tomography as an imaging biomarker in a prospective, longitudinal cohort of patients with large vessel vasculitis. Arthritis Rheumatol. 2017.Google Scholar
- 65.Salvarini C, Macchioni PL, et al. Polymyalgia rheumatic and giant cell arteritis : 5 year epidemiologic and clinical study Reggio Emilia, Italy. Clin Exp Rheum. 1987;5:205.Google Scholar
- 67.Hunder GG. Giant cell arteritis and polymyalgia rheumatica. In: Kelly WN, Harris ED, editors. Textbook of rheumatology. 5th ed. Philadelphia: WB Saunders; 1996.Google Scholar
- 69.Mazlumzadeh M, Hunder GG, Easley KA, Calamia KT, Matteson EL, Griffing WL, et al. Treatment of giant cell arteritis using induction therapy with high dose glucocorticoids: a double-blind, placebo controlled, randomized prospective clinical trial. Arthritis Rheum. 2006;54:3310–8.CrossRefPubMedGoogle Scholar
- 72.Spiera RF, Mitnick HJ, et al. A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis. Clin Exp Rheum. 2001;19:495.Google Scholar
- 76.•• Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of tocilizumab in giant cell arteritis. N Engl J Med. 2017;377:317–28. This study provided the knowledge necessary to lead to the first FDA approved treatment as steroid sparing agent in GCA. CrossRefPubMedGoogle Scholar