Full Papers
The Joint Vasculitis Registry in German-speaking countries (GeVas): subgroup analysis of 266 AAV patients
S. Arnold1, P. Wallmeier2, A. Tais3, G. Ihorst4, M. Janoschke5, F. Schubach6, P. Aries7, R. Bergner8, J.P. Bremer9, N. Görl10, E. Gutdeutsch11, B. Hellmich12, J. Henes13, B.F. Hoyer14, A. Kangowski15, I. Kötter16, M. Krusche17, T. Magnus18, C. Metzler19, U. Müller-Ladner20, J. Petersen21, A. Reichelt De Tenorio22, M. Schaier23, J.H. Schirmer24, U. Schönermarck25, J. Thiel26, L. Unger27, N. Venhoff28, J. Weinmann-Menke29, C. Iking-Konert30, P. Lamprecht31
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany. sabrina.arnold@uksh.de
- Department of Nephrology, Asklepios Klinikum Barmbek, Hamburg; and III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
- Clinical Trials Unit, Medical Centre, Faculty of Medicine, University of Freiburg, Germany.
- Clinical Trials Unit, Medical Centre, Faculty of Medicine, University of Freiburg, Germany.
- Clinical Trials Unit, Medical Centre, Faculty of Medicine, University of Freiburg, Germany.
- Interdisciplinary Medical Intensive Care, Medical Centre, Faculty of Medicine, University of Freiburg, Germany.
- Immunologikum, Department of Rheumatology, Hamburg, Germany.
- Klinikum Ludwigshafen, Department of Internal Medicine, Haemato-oncology, Nephrology, Infectiology and Rheumatology, Ludwigshafen am Rhein, Germany.
- Immunologikum, Department of Rheumatology, Hamburg, Germany.
- Klinikum Südstadt Rostock, Department of Internal Medicine, Rheumatology and Immunology, Rostock, Germany.
- Department of Rheumatology, Barmherzige Brüder Regensburg, Germany.
- Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabetology, Kirchheim unter Teck, Germany.
- University Medical CentreTübingen, Centre for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases and Department of Internal Medicine II, Tübingen, Germany.
- Department of Rheumatology, UKSH Campus Kiel, Germany.
- Klinikum Südstadt Rostock, Department of Internal Medicine, Rheumatology and Immunology, Rostock, Germany.
- III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg; and Department of Rheumatology and Immunology, Klinikum Bad Bramstedt, Germany.
- III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
- Department of Neurology, University Medical Center Hamburg-Eppendorf, Germany.
- Immunologikum, Department of Rheumatology, Hamburg, and Department of Internal Medicine and Rheumatology, Klinikum Bielefeld, Germany.
- Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany.
- Department of Rheumatology and Immunology, Klinikum Bad Bramstedt, and MEDIZINICUM, Hamburg, Germany.
- Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Department of Internal Medicine, Rheumatology, Pulmonology, Nephrology and Diabetology, Kirchheim unter Teck, Germany.
- Department of Nephrology, University Medical Centre Heidelberg, Germany.
- Department of Rheumatology, UKSH Campus Kiel, Germany.
- Department of Medicine IV, Nephrology Division, LMU University Hospital, LMU Munich, Germany.
- Clinical Department of Rheumatology and Immunology, University Medical Centre Graz, Austria.
- Medical Department 1, Städtisches Klinikum Dresden, Germany.
- Department of Rheumatology and Clinical Immunology, Medical Centre, Faculty of Medicine, University of Freiburg, Germany.
- Department of Nephrology, University Medical Centre of the Johannes Gutenberg University, Mainz, Germany.
- III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; and Department of Rheumatology, Stadtspital Zürich, Switzerland.
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
CER17210
2024 Vol.42, N°4
PI 0852, PF 0858
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PMID: 38607682 [PubMed]
Received: 18/10/2023
Accepted : 08/01/2024
In Press: 12/04/2024
Published: 29/04/2024
Abstract
OBJECTIVES:
Prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany to date. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. The aim of this study is to present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry.
METHODS:
GeVas is a prospective, web-based, multicentre, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis. Recruitment started in June 2019.
RESULTS:
Between June 2019 and October 2022, 266 patients with AAV were included in the GeVas registry: 173 (65%) with new-onset and 93 (35%) with relapsing AAV. One hundred and sixty-two (61%) patients were classified as granulomatosis with polyangiitis (GPA), 66 (25%) as microscopic polyangiitis (MPA), 36 (13%) as eosinophilic granulomatosis with polyangiitis (EGPA), and 2 (1%) as renal limited AAV. The median age was 59 years (51-70 years, IQR), 130 (51%) patients were female. Most patients were ANCA positive (177; 67%) and affected by general symptoms, pulmonary, ear nose throat (ENT), renal and neurological involvement. For induction of remission, the majority of patients received glucocorticoids (247, 93%) in combination with either rituximab (118, 45%) or cyclophosphamide (112, 42%).
CONCLUSIONS:
Demographic characteristics are comparable to those in other European countries. Differences were found regarding ANCA status, frequencies of organ manifestations, and therapeutic regimens. The GeVas registry will allow longitudinal observations and prospective outcome measures in AAV.