Search

ObjectivesInfection exerts a major burden in ANCA-associated vasculitis (AAV), however, its precise extent and nature remains unclear. In this national study we aimed to longitudinally quantify, characterize and contextualize infection risk in AAV.MethodsWe conducted a multicentre matched cohort study of AAV. Complementary data on infections were retrieved via data linkage with the population-based Scottish microbiological laboratory, hospitalization and primary care prescribing registries.ResultsA total of 379 AAV patients and 1859 controls were followed up for a median of 3.5 years (interquartile range 1.9–5.7). During follow-up, the proportions of AAV patients with at least one laboratory-confirmed infection, severe infection and primary care antibiotic prescription were 55.4%, 35.6% and 74.6%, respectively. The risk of infection was higher in AAV than in matched controls {laboratory-confirmed infections: incidence rate ratio [IRR] 7.3 [95% confidence interval (CI) 5.6, 9.6]; severe infections: IRR 4.4 [95% CI 3.3, 5.7]; antibiotic prescriptions: IRR 2.2 [95% CI 1.9, 2.6]}. Temporal trend analysis showed that AAV patients remained at a higher risk of infections throughout the follow-up period, especially year 1. Although the Escherichia genus was the most commonly identified pathogen (16.6% of AAV, 5.5% of controls; P ConclusionAAV patients have up to seven times higher risk of infection than the general population and the overall risk remains significant after 8 years of follow-up. The testing of enhanced short- to medium-term prophylactic antibiotic regimes should be considered.

Objectives : To study variations in Canadian clinical practice patterns for the management of ANCA-associated vasculitis (AAV) and identify points to consider for the development of national recommendations. Material and Methodology : A 30-item needs assessment questionnaire was sent to all members of the Canadian Vasculitis network (CanVasc), Canadian Rheumatology Association (CRA), Canadian Thoracic Society (CTS) and Canadian Society of Nephrology (CSN). Respondent characteristics, practice patterns, concerns and expectations were analyzed. Results : Among 132 physicians who followed at least 1 vasculitis patient and responded to the survey, 39% stated that they felt confident in their management of AAV. Several variations in practice were observed regarding diagnostic procedure, induction and maintenance treatments and use of biologics; some were due to logistic constraints (difficulties in access to some specific tests, drugs or care; lack of health care coverage for the costs). The top 5 topics for which recommendations are expected involve treatment for remission induction, maintenance, refractory disease, and relapse as well as biologics. Conclusion : Practice variations identified in this needs assessment survey will serve to formulate key questions for the development of CanVasc recommendations.

The Canadian Vasculitis research network (CanVasc) is composed of physicians from different medical specialties, including rheumatology and nephrology and researchers with expertise in vasculitis. One of its aims was to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides in Canada. This executive summary features the 19 recommendations and 17 statements addressing general AAV diagnosis and management, developed by CanVasc group based on a synthesis of existing international guidelines, other published supporting evidence and expert consensus considering the Canadian healthcare context.

Objective.To determine frequency and outcomes of granulomatosis with polyangiitis (GPA)–related cardiac disease in a North American GPA cohort.Methods.Analysis was done of all patients in the Vasculitis Clinical Research Consortium Longitudinal Study of GPA. Demographic and clinical characteristics of patients with and without GPA-related cardiac involvement were compared.Results.Of 517 patients with GPA, 3.3% had cardiac involvement. No differences were observed between patients with or without cardiac involvement in terms of demographics, antineutrophil cytoplasmic antibody positivity, or relapse rate.Conclusion.Cardiac involvement in GPA is rare and heterogeneous. In this cohort, cardiac involvement was not associated with a higher rate of relapse or premature death.

Temporal arteritis in the form of giant cell arteritis (GCA) is common in the elderly but is extremely rare in patients less than 50 years of age. We describe two male patients: one who presented at the age of 31 years with painful, nodular swellings of both temporal arteries and whose temporal artery biopsy demonstrated a non-giant cell panarteritis with mixed inflammatory cell infiltrate typical of juvenile temporal arteritis (JTA); another one, aged 40 years, who presented with headache and cerebral angiography consistent with an intracranial vasculitis and whose temporal artery biopsy confirmed an authentic multinucleated GCA. The first patient spontaneously improved after biopsy and the second patient has responded well to corticosteroid therapy. These two cases exemplify well two distinct but extremely rare forms of temporal arteritis in young patients. A 3rd subset is that associated with a systemic vasculitis. Few cases of JTA have been reported and, to our knowledge, we describe in this report one of the only cases of GCA with central nervous system involvement in the young.