1 Nesher, et al: PSV incidence in Jerusalem Personal non-commercial use only. The Journal of Rheumatology Copyright © 2016. All rights reserved. The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study Gideon Nesher, Eli Ben-Chetrit, Bracha Mazal, and Gabriel S. Breuer ABSTRACT. Objective. The incidence of primary systemic vasculitides varies among different geographic regions and ethnic origins. The aim of this study was to examine the incidence rates of vasculitides in the Jerusalem Jewish population, and to examine possible trends in incidence rates over a 20-year period. Methods. The clinical databases of inpatients at the 2 medical centers in Jerusalem were searched for patients with vasculitis diagnosed between 1990–2009. Individual records were then reviewed by one of the authors. The significance of trends in incidence rates throughout the study period was evaluated by Pearson correlation coefficient. Results. The average annual incidence rate of polyarteritis nodosa was 3.6/million adults (95% CI 1.6–4.7). Incidence rates did not change significantly during this period (r = 0.39, p = 0.088). The incidence of granulomatosis with polyangiitis (GPA) was 4.1 (2.2–5.9) for the whole period, during which it increased significantly (r = 0.53, p < 0.05). The incidence of microscopic polyangiitis (MPA) was lower: 2.3 (1.2–3.5)/million. It also increased significantly (r = 0.55, p < 0.05). The incidence of eosinophilic granulomatosis with polyangiitis was 1.2 (0.4–1.9), which remained stable throughout the study period. The incidence of Takayasu arteritis was 2.1/million (95% CI 1.2–2.9), and it also remained stable. Giant cell arteritis (GCA) incidence was 8.1 (5.7–10.6)/100,000 population aged 50 years or older. In sharp contrast with other vasculitides, its incidence decreased significantly throughout the study period (r = –0.61, p < 0.01). Conclusion. The incidence rates of vasculitides in the Jewish population of Jerusalem are in the lower range of global incidence rates. While GPA and MPA incidence are increasing, GCA incidence is decreasing. (J Rheumatol First Release April 15 2016; doi:10.3899/jrheum.150557) Key Indexing Terms: VASCULITIS MICROSCOPIC POLYANGIITIS ISRAEL GRANULOMATOSIS WITH POLYANGIITIS GIANT CELL ARTERITIS INCIDENCE From the Department of Internal Medicine, Rheumatology Unit and Infectious Disease Unit, Shaare-Zedek Medical Center; Hebrew University Medical School; the Rheumatology Unit, Hadassah Medical Center, Jerusalem, Israel. G. Nesher, MD, Clinical Associate Professor, Department of Internal Medicine and the Rheumatology Unit, Shaare-Zedek Medical Center, and the Hebrew University Medical School; E. Ben-Chetrit, MD, Department of Internal Medicine and the Infectious Disease Unit, Shaare-Zedek Medical Center, and Lecturer, Hebrew University Medical School; B. Mazal, MD, the Rheumatology Unit, Hadassah Medical Center; G.S. Breuer, MD, Clinical Senior Lecturer, Department of Internal Medicine and the Rheumatology Unit, Shaare-Zedek Medical Center, and Hebrew University Medical School. Address correspondence to Dr. G.S. Breuer, Head, Rheumatology Unit, Shaare-Zedek Medical Center, PO Box 3235, Jerusalem, Israel 91031. E-mail: gbreuer@szmc.org.il Accepted for publication January 12, 2016. The primary systemic vasculitides (PSV) are a heterogeneous group of inflammatory diseases of unknown etiology, affecting blood vessels of different sizes. The incidence of PSV varies among different geographic regions and ethnic origins. Giant cell arteritis (GCA) is more common in northern countries 1,2,3 , and Takayasu arteritis (TA) is considered more common in the Far East. Among the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV), granulomatosis with polyangiitis (GPA) tends to be more common in Europeans, whereas micro- scopic polyangiitis (MPA) tends to be relatively more common in Japan and other Asian countries, and in Kuwait 4,5,6 . The incidence of polyarteritis nodosa (PAN) was reported to be exceptionally high in native Alaskans and in Kuwait, compared to reports from European countries 6,7,8,9,10 . These differences may be due to both ethnic and environmental factors 4 . The Jewish people in Israel are unique in this regard: origi- nating in the Middle East, they were dispersed in Europe after being conquered by the Roman Empire about 2000 years ago, and later spread mainly to North Africa and parts of the Middle East. They were largely conserved genetically with some local genetic influences throughout those centuries 11 . Upon emigrating to Israel during the last century, intermar- riages between ethnic groups were initially uncommon, but became increasingly common in the last decades. Therefore those genetic pools are mixed mainly in the younger population. www.jrheum.org Downloaded on January 23, 2022 from