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To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden.Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register. The study area is Skåne, the southernmost county of Sweden (population 1.29 million; 21.4% aged18 years). Case records for children (0-17 years) assigned a diagnosis code between M300 and M319 and/or D690 were reviewed to ascertain diagnosis. Only patients diagnosed between 2004 and 2014 were included.In total, 556 patients with PSV were identified. The annual incidence rate per million children (95% confidence interval) was estimated to be 200 (183-217) for all PSV, 175.5 for IgAV (160-191), 20.1 for KD (14.9-25.4), 1.4 (0-2.8) for each of GPA and MPA, 0.7 (0-1.7) for PAN, and 0.4 (0-1.1) for each of EGPA and TAK. Among children aged10 years, 99.5% of cases were either IgAV or KD, both exhibiting a seasonal pattern paralleling infections. There were no deaths, but three cases of end-stage renal disease were noted, all in MPA.Vasculitis is relatively common during childhood. Mild cases associated with the infection season are most common in the youngest age groups, while during adolescence a substantial proportion has more severe forms of vasculitis.

Giant cell arteritis (GCA) is a systemic disease with extensive vascular involvement. The aim of this study was to investigate the cumulative incidence of large vessel involvement (LVI) in GCA, the distribution of vessels involved, and predictors for LVI.Patients with biopsy-proven GCA in a defined area in southern Sweden, diagnosed between 1997 and 2010, were identified through the register of the regional Department of Clinical Pathology. A structured review of all medical records and imaging and histopathology reports was performed. Imaging studies for an age- and sex-matched reference cohort were also reviewed.A total of 164 patients with GCA were investigated, of whom 24 (15%) had LVI. LVI manifestations were detected a median of 3.7 [interquartile range (IQR) 0.7-7.5] years after GCA diagnosis. Aortic involvement was found in 16 patients (10%), mainly aneurysms/ectasias of the thoracic aorta. Two patients had aortic dissections. Fourteen patients had tributary involvement. In the reference population, the cumulative incidence of LVI overall was 10.8% and aortic involvement was found in 5.4%. The presence of giant cells in the biopsy was significantly less frequent among GCA patients with LVI (23% vs. 52%; p = 0.01), and a presentation with polymyalgia rheumatica (PMR) was more frequent (44% vs. 20%, p = 0.01).The estimated incidence of LVI, detected by imaging in a clinical setting, was higher among patients with GCA than the reference population. The aorta was the most commonly affected vascular territory. The negative association with giant cells may suggest particular mechanisms in this subset of GCA.

Objectives: To study the association between late organ damage in patients with primary systemic vasculitis (PSV) and cigarette smoking. PSV included Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS), and polyarteritis nodosa (PAN). Methods: The pattern and extent of organ damage according to the Vasculitis Damage Index (VDI) were analysed for 86 prevalent cases with PSV retrieved from a geographically defined population in southern Sweden (46 WG, 27 MPA, four CSS, and nine PAN). Data on clinical findings, laboratory tests, and smoking habits were collected from case records from the time of diagnosis. The patients were stratified into two main groups according to their smoking habits: smokers (subdivided into active and ex-smokers) and non-smokers (patients who had never smoked). Results: Data on smoking habits were available for 77 patients (90%). Thirty-three (38%) patients were categorized as smokers and 44 (51%) were non-smokers. Smoking was more common in men (61.5% vs. 23.6% in women, p = 0.001). There were no differences in smoking habits between the main diagnostic groups (WG 40% smokers, MPA 45%). Ear, nose, and throat (ENT) damage was significantly more prevalent in non-smokers (p = 0.001). Myocardial infarction (MI) and end-stage renal disease (ESRD) were more common in the current smokers (p = 0.04) than in the non-smokers. Conclusions: We found ENT damage to be significantly less prevalent in smokers. This is the first report of a possible modifying effect of cigarette smoking on the development of organ damage in PSV, but more studies are needed before any firm conclusions can be made.