Giant Cell Arteritis: The Experience of Two Collaborative Referral Centers and an Overview of Disease Pathogenesis and Therapeutic Advancements

Rosanna Dammacco; Giovanni Alessio; Ermete Giancipoli; Patrizia Leone; Anna Cirulli; Leonardo Resta; Angelo Vacca; Franco Dammacco

doi:10.2147/OPTH.S243203

Giant Cell Arteritis: The Experience of Two Collaborative Referral Centers and an Overview of Disease Pathogenesis and Therapeutic Advancements

Clin Ophthalmol. 2020 Mar 11:14:775-793. doi: 10.2147/OPTH.S243203. eCollection 2020.

Authors

Rosanna Dammacco¹, Giovanni Alessio¹, Ermete Giancipoli², Patrizia Leone³, Anna Cirulli³, Leonardo Resta⁴, Angelo Vacca³, Franco Dammacco³

Affiliations

¹ Department of Ophthalmology and Neuroscience, University of Bari "Aldo Moro", Medical School, Bari, Italy.
² Department of Biomedical Sciences, Ophthalmology Unit, University of Sassari, Sassari, Italy.
³ Department of Biomedical Sciences and Human Oncology, University of Bari "Aldo Moro", Medical School, Bari, Italy.
⁴ Department of Emergency and Organ Transplantation, University of Bari "Aldo Moro", Medical School, Bari, Italy.

Abstract

Purpose: Giant cell arteritis (GCA), a chronic vasculitis of the large and medium-sized arteries, affects people >50 years of age. This study assessed the prevalence of visual manifestations and other clinical features at presentation in an Italian cohort of GCA patients. Recent advances in the pathophysiology, diagnosis, and therapy of GCA are also reviewed.

Methods: This retrospective, single-center study conducted by the ophthalmology and internal medicine clinics of one university recruited 56 patients from 2005 to 2016 and followed them for 11-54 months.

Results: Ocular involvement was diagnosed in 19 patients (33.9%), with permanent vision loss in 19.6% (7.1% of the cohort with bilateral vision loss). Arteritic anterior and posterior ischemic optic neuropathy were diagnosed in 11 patients (57.9%) and 1 patient (5.3%), respectively, cotton wool spots in 3 patients (15.8%), central retinal artery occlusion in 2 patients (10.5%), and anterior segment ischemia and multifocal choroidal ischemia in 1 patient each (5.3%). Polymyalgia rheumatica was associated with GCA in 44.6% of the patients. The most common extra-ocular manifestation was constitutional symptoms (82.1% of the patients). Large-vessel involvement, including of the ascending aorta, aortic arch, and left axillary artery, was diagnosed by magnetic resonance or computed tomography (CT) angiography and ¹⁸FDG positron emission/CT. Glucocorticoids (GCs) remain the standard-of-care worldwide, but methotrexate, provided as a steroid-sparing drug in 41% of the patients, resulted in earlier tapering, a lower cumulative dose of GCs, and a lower rate of relapse. Among the combinations of GCs and immunosuppressive drugs proposed to treat GCA, only tocilizumab has effectively induced and maintained disease remission.

Conclusion: According to our data and literature reports: a) GCA is a systemic disease; b) its diagnosis is expedited by the adjunct use of imaging techniques; c) insights into the pathogenesis of GCA may allow an improved, differentiated therapeutic approach.

Keywords: diagnostic imaging; giant cell arteritis; glucocorticoids; ocular manifestations; pathogenetic advances; tocilizumab.

Grants and funding

This work was supported by Fondo di Sviluppo e Coesione 2007-2013 – APQ Ricerca Regione Puglia “Programma regionale a sostegno della specializzazione intelligente e della sostenibilità sociale ed ambientale - FutureInResearch”. The sponsors of this study are public or nonprofit organizations that support science in general; they had no role in gathering, analyzing, or interpreting the data.