Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

Abstract

Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence Electronic supplementary material The online version of this article (

Granulomatosis with Polyangiitis and Nasal Involvement - What Radiological Markers Point the Disease

“…Patients with GPA in study group showed higher prevalence of ENT involvement than described by POLVAS registry (90,5%vs75,5%). The rest of organ involvement presented on a lower level according to the registry except for the neurological involvement (peripheral nervous system) -in our study group it was 24,5% compared to 18% in POLVAS registry [3].…”

“…The disease involves small or small and medium-sized vessels. Its prevalence in Europe is 23 to 160 people per 1 million and affects primarily adult caucasians (rarely children), with no gender predilection [2,3]. Polish Vasculitis Registry encompassing 58.2% of Polish population showed that demographic characteristics and frequency of AAV in Poland is similar to other European countries [3].…”

“…According to a retrospective analysis of POLVAS registry, in AAV patients, independent risk factors for death are permanent renal replacement therapy (PRRT), kidney involvement/respiratory involvement, age > 65 years, and the presence of c/PR3 ANCA. Ear, nose and throat (ENT) involvement was identified a factor negatively associated with PRRT [3].…”

Association of antineutrophil cytoplasmic antibody (ANCA) specificity with the demographic and clinical characteristics of patients with ANCA-associated vasculitides

“…These prognosticators are older age (>65 years) and renal involvement, and they are included in the modified Five-Factor Score as parameters predictive of prognosis in systemic necrotizing vasculitides [15]. This refers to MPO-ANCA-positive EGPA but even more so to other MPO-ANCA-positive vasculitides, mainly MPA [2,12].…”

“…Their clinical and laboratory data were collected in POLVAS registry by 9 referral centers (14 clinical wards) from 7 Voivodships with 22,3 mln inhabitants, encompassing 58.2% of the Polish population between March 2016 and January 2017. Specialties of the centers included rheumatology (10), nephrology (2), metabolic diseases (1) and clinical immunology (1) [12]. We only included individuals who met the American College of Rheumatology classification criteria for Wegener granulomatosis (presently GPA) or Churg-Strauss syndrome (presently EGPA) as well as criteria according to the nomenclature of GPA, MPA, and EGPA proposed by the 2012 Chapel Hill Consensus Conference (CHCC 2012) [1] regardless of their disease activity at the entry.…”