Stable incidence of primary systemic vasculitides over five years: Results from the German vasculitis register

• Published in: Arthritis and rheumatism Vol. 53; no. 1; pp. 93 - 99
• Main Authors: Reinhold‐Keller, Eva, Herlyn, Karen, Wagner‐Bastmeyer, Rosemarie, Gross, Wolfgang L.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 15.02.2005
• Subjects: Adult; Age Distribution; Aged; Aged, 80 and over; ANA‐associated vasculitides; Churg-Strauss Syndrome - epidemiology; Epidemiology; Female; Germany - epidemiology; Granulomatosis with Polyangiitis - epidemiology; Humans; Incidence; Male; Middle Aged; Primary systemic vasculitides; Prospective Studies; Registries; Sex Distribution; Vasculitis; Wegener's granulomatosis; Germany
• ISSN: 0004-3591; 1529-0131
• DOI: 10.1002/art.20928

Objective To register all newly diagnosed patients with primary systemic vasculitides (PSV) in a large region in northern Germany. Methods Between January 1, 1998 and December 31, 2002 all newly diagnosed cases of PSV were identified in a large mixed rural/urban northern German region consisting of 2,777,275 habitants in a population‐based prospective study. The following sources were used: departments of all hospitals, including their outpatient clinics; all departments of pathology; and the reference immunologic labs serving the catchment area. Results During 5 years, 642 PSV patients were identified. The incidence rates for all PSV were between 40 and 54 cases per 1 million and per year. People at age 50 years and older had a 3–5‐fold higher risk of developing PSV compared with those younger than 50 years. The incidence rates of antineutrophil cytoplasmic antibody (ANCA)–associated PSV (Wegener's granulomatosis [WG], microscopic polyangiitis [MPA], Churg‐Strauss syndrome [CSS]) were between 9.5 and 16/million/year. WG occured 2–3 times more frequently than MPA or CSS. Conclusion Results of a population‐based vasculitis register over 5 years for the incidence of PSV among 2.78 million habitants in northern Germany revealed a stable incidence for all PSV. Compared with other European studies coming from small regions or referral centers, the incidence rates for ANCA‐associated PSV were the same as in Norway, lower than those in United Kingdom, but higher than those in Spain.