Affordable Access

deepdyve-link
Publisher Website

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

Authors
  • Wójcik, K1
  • Wawrzycka-Adamczyk, K1
  • Włudarczyk, A1
  • Sznajd, J2, 3
  • Zdrojewski, Z4
  • Masiak, A4
  • Czuszyńska, Z4
  • Majdan, M5
  • Jeleniewicz, R5
  • Klinger, M6
  • Jakuszko, K6
  • Rowaiye, O6
  • Brzosko, M7
  • Milchert, M7
  • Dębska-Ślizień, A8
  • Storoniak, H8
  • Tłustochowicz, W9
  • Kur-Zalewska, J9
  • Wisłowska, M10
  • Madej, M11
  • And 4 more
  • 1 2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, ul. Skawińska 8, 31-066, Kraków, Poland. , (Poland)
  • 2 Department of Rheumatology, Raigmore Hospital, Inverness, UK.
  • 3 Institute of Applied Health Sciences, University of Aberdeen, Aberdeen, UK.
  • 4 Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Gdańsk, Poland. , (Poland)
  • 5 Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Lublin, Poland. , (Poland)
  • 6 Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Wrocław, Poland. , (Poland)
  • 7 Department of Rheumatology and Internal Diseases, Pomeranian Medical University in Szczecin, Szczecin, Poland. , (Poland)
  • 8 Department of Nephrology, Transplantology and Internal Diseases, Medical University of Gdańsk, Gdańsk, Poland. , (Poland)
  • 9 Department of Internal Medicine and Rheumatology, Military Medicine Institute, Warszawa, Poland. , (Poland)
  • 10 Department of Internal Diseases and Rheumatology, Central Clinical Hospital of the Ministry of the Interior and Administration, Warszawa, Poland. , (Poland)
  • 11 Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wrocław, Poland. , (Poland)
  • 12 Department of Internal Medicine and Metabolic Diseases, Medical University of Silesia, Katowice, Poland. , (Poland)
  • 13 Department of Rheumatology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warszawa, Poland. , (Poland)
  • 14 Department of Internal Medicine and Rheumatology, Medical University of Silesia, Katowice, Poland. , (Poland)
  • 15 2nd Department of Internal Medicine, Faculty of Medicine, Jagiellonian University Medical College, ul. Skawińska 8, 31-066, Kraków, Poland. [email protected] , (Poland)
Type
Published Article
Journal
Clinical Rheumatology
Publisher
Springer-Verlag
Publication Date
Sep 01, 2019
Volume
38
Issue
9
Pages
2553–2563
Identifiers
DOI: 10.1007/s10067-019-04538-w
PMID: 31016580
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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). 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). 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 interval (CI) = 2.3-12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06-9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05-6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3-25.5), skin involvement (OR = 4.4; 95% CI = 1.02-19.6), and age > 65 (OR = 6.3; 95% CI = 1.18-33.7). In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.

Report this publication

Statistics

Seen <100 times