Reuma.pt/vasculitis – the Portuguese vasculitis registry

Abstract

Background: The vasculitides are a group of rare diseases with different manifestations and outcomes. New therapeutic options have led to the need for long-term registries. The Rheumatic Diseases Portuguese Register, Reuma.pt, is a web-based electronic clinical record, created in 2008, which currently includes specific modules for 12 diseases and > 20,000 patients registered from 79 rheumatology centres. On October 2014, a dedicated module for vasculitis was created as part of the European Vasculitis Society collaborative network, enabling prospective collection and central storage of encrypted data from patients with this condition. All Portuguese rheumatology centres were invited to participate. Data regarding demographics, diagnosis, classification criteria, assessment tools, and treatment were collected. We aim to describe the structure of Reuma.pt/vasculitis and characterize the patients registered since its development. Results: A total of 687 patients, with 1945 visits, from 13 centres were registered; mean age was 53.4 ± 19.3 years at last visit and 68.7% were females. The most common diagnoses were Behçet's disease (BD) (42.5%) and giant cell arteritis (GCA) (17.8%). Patients with BD met the International Study Group criteria and the International Criteria for BD in 85.3 and 97.2% of cases, respectively. Within the most common small-and medium-vessel vasculitides registered, median [interquartile range] Birmingham Vasculitis Activity Score (BVAS) at first visit was highest in patients with ANCA-associated vasculitis (AAV) (17.0 [12.0]); there were no differences in the proportion of patients with AAV or polyarteritis nodosa who relapsed (BVAS≥1) or had a major relapse (≥1 major BVAS item) during prospective assessment (p = 1.00, p = 0.479). Biologic treatment was prescribed in 0.8% of patients with GCA, 26.7% of patients with AAV, and 7.6% of patients with BD. There were 34 (4.9%) deaths reported.

One year in review 2021: Behçet's syndrome

“…The women to men ratio was 2.2 to 1. A vasculitis registry from Portugal including patients from 13 centres showed that BS is the most common vasculitis in this registry (298/687, 42.5%), despite the longest diagnostic delay (82.5±110.1 months) (6). The authors suggest that a new prevalence study of BS in Portugal is warranted, as the 2 previous prevalence studies showing 1.5 per 100,000 in 1991 (7) and 2.4 per 100,000 in 1997 (8) may not be reflecting the true prevalence.…”

“…Portugal and a primary care database from UK, suggested a higher prevalence of BS than previously reported from these countries (6,9). • A study from Egypt suggested that BS has a more severe course among men (11).…”

Neues zum Behçet-Syndrom

“…Eine 2020 veröffentlichte Auswertung eines portugiesischen Vaskulitis-Registers konnte herausarbeiten, dass das BS die häufigste Vaskulitis überhaupt darstellt (298 von insgesamt 687 Patienten, entsprechend 42,5 %). In Kenntnis früher genannter Prävalenzen zwischen 1,5 und 2,4 pro 100000 diskutierten die Autoren die Notwendigkeit, dass neue Studien zur Erfassung der BS-Prävalenz in Portugal dringend erforderlich seien [5].…”

A glance into the future of anti-neutrophil cytoplasmic antibody-associated vasculitis

“…]);)]; Germany, Austria, and Switzerland [Joint Vasculitis Registry in German-speaking countries (GeVas)]; Greece (AAV Patient Registry); USA and Canada [Vasculitis Patient-Powered Research Network (VPPRN)]. 121 , 202 – 215 However, these existing registries exhibit significant differences in terms of content, data collection (retrospective vs prospective), stages of development, and aims, primarily influenced by the medical specialties involved (e.g. nephrology, rheumatology, internal medicine, immunology) and local resources.…”

“…Therefore, registries are a vital source of clinical data and can be particularly useful in facilitating research in disease biomarkers, optimizing recruitment for clinical trials, and deepening our understanding of the natural course of AAV. 201 121,[202][203][204][205][206][207][208][209][210][211][212][213][214][215] However, these existing registries exhibit significant differences in terms of content, data collection (retrospective vs prospective), stages of development, and aims, primarily influenced by the medical specialties involved (e.g. nephrology, rheumatology, internal medicine, immunology) and local resources.…”