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OBJECTIVES To estimate the point prevalence (p.p.) of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) within a defined population in southern Sweden. METHOD A cross-sectional p.p. study using multiple sources for case identification. The study area, a healthcare district around the(More)
OBJECTIVE To study the incidence, prevalence and clinical characteristics of Behçet's disease (BD) in a defined population in southern Sweden. METHODS The study area consists of three health-care districts with an adult population (≥15 years) of 809 317 on 1 January 2011 (25% of non-Swedish ancestry), situated in Skåne, the southernmost county in Sweden.(More)
OBJECTIVES To study the clinical characteristics and epidemiology of the combination of primary systemic vasculitis (PSV) and severe alpha-1 antitrypsin (α1-AT) deficiency. METHOD Patients with PSV [granulomatosis with polyangiitis (GPA) (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), and(More)
OBJECTIVES Polyarteritis nodosa (PAN) is a term that has been used to describe a wide variety of vasculitic conditions. In 1994, the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitides proposed that the name classical PAN should be restricted to diseases where there is arteritis in small and medium-sized arteries without the(More)
OBJECTIVES To assess the extent and pattern of irreversible organ damage in patients with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN), and Churg-Strauss syndrome (CSS) by a cross-sectional point prevalence study within a defined geographical area. METHODS The Vasculitis Damage Index (VDI) was recorded for 86(More)
OBJECTIVE To estimate the incidence of and survival rates for WG, microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and PAN within a defined population in southern Sweden. METHODS Cases were retrieved using hospital records and a serology database. All new cases of WG, MPA, CSS and PAN between 1997 and 2006 were included, provided they met(More)
BACKGROUND AND OBJECTIVES ANCA-associated vasculitis is commonly found in elderly patients, but there are few data concerning outcome and treatment in the highest age groups. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Consecutive patients (N=151) presenting between 1997 and 2009 were retrospectively included from local registries in six centers in(More)
It is now 25 years since the first European studies on vasculitis--the anti-neutrophil cytoplasmic antibody (ANCA) standardization project. Over that period of time, there have been major developments in the classification of the vasculitides, which has permitted the conduct of high-quality epidemiology studies. Studying the epidemiology of rare diseases(More)
BACKGROUND Involvement of large arteries is well-documented in giant-cell arteritis (GCA), but the risk for cardiovascular events is not well-understood. OBJECTIVE To evaluate the risks for incident myocardial infarction (MI), cerebrovascular accident (CVA), and peripheral vascular disease (PVD) in individuals with incident GCA in a general population(More)
BACKGROUND Conventional treatment of eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss) with glucocorticoids, with or without additional immunosuppressive drugs, is limited by partial efficacy, frequent toxicity and a high relapse rate. Rituximab is a licensed treatment for granulomatosis with polyangiitis and microscopic polyangiitis and(More)