Morbidity and Mortality of Large-Vessel Vasculitides

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RECENT ADVANCES IN LARGE VESSEL VASCULITIS ( C DEJACO AND C DUFTNER, SECTION EDITORS)

Morbidity and Mortality of Large-Vessel Vasculitides Matthew J. Koster 1 & Kenneth J. Warrington 1 & Eric L. Matteson 1

# Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Purpose of Review Giant cell arteritis (GCA) and Takayasu arteritis (TAK) comprise the primary systemic large-vessel vasculitides. In these conditions, arterial stenosis, occlusion, aneurysm, and dissection can lead to severe disease-related consequences. This review focuses on disease-related manifestations of GCA and TAK, emphasizing the impact of these findings on long-term morbidity and mortality. Recent Findings Vision loss remains a main contributor of morbidity in GCA. Non-invasive imaging allows for recognition of aortic disease in GCA but monitoring and intervention guidelines require further development. TAK represents a severe disease of early-onset with high risk of morbidity due to aortic, pulmonary, cardiovascular, and neurologic involvement. Overall, patients with GCA have similar mortality rates to comparators but mortality is notably higher than the general population in TAK. Summary A multidisciplinary approach of expert subspecialists is required to assist with the complex care of patients with GCA and TAK in order to appropriately surveil, identify, and address the multi-faceted co-morbidities of these diseases. Keywords Giant cell arteritis . Takayasu arteritis . Large-vessel vasculitis . Morbidity . Mortality . Outcome

Introduction

Giant Cell Arteritis

The primary large-vessel vasculitides are giant cell arteritis (GCA) and Takayasu arteritis (TAK). Both conditions produce granulomatous, transmural inflammation of the aorta and its major branches, resulting in several overlapping clinical and radiographic features. Despite their similarities, notable differences exist, particularly age of onset and arterial segment predilection. Consequently, the morbidity and mortality associated with these two conditions differ considerably. This review highlights the common disease-related morbidities associated with GCA and TAK and provides current understanding of the mortality risk in these conditions.

Visual Ischemia

This article is part of the Topical Collection on Recent Advances in Large Vessel Vasculitis * Matthew J. Koster [email protected] 1

Department of Internal Medicine, Division of Rheumatology, Mayo Clinic College of Medicine and Science, 200 1st Street SW, Rochester, MN 55905, USA

Vision loss is among the most-feared morbidities of GCA and all acute ischemic ophthalmologic manifestations of GCA are considered emergencies due to risk of either progression or permanence. The most common cause of visual ischemia in GCA is vasculitic narrowing or occlusion of the ophthalmic artery and its branches, particularly the central retinal artery and the posterior ciliary arteries [1]. Arteritic anterior ischemic optic neuropathy is major cause of blindness in GCA (80– 90% of cases), but central retinal artery