Diagnosis
Subphenotypes of ANCA-associated vasculitis identified by latent class analysis
K. Wójcik1, G. Biedroń2, K. Wawrzycka-Adamczyk3, S. Bazan-Socha4, A. Ćmiel5, Z. Zdrojewski6, A. Masiak7, Z. Czuszyńska8, M. Majdan9, R. Jeleniewicz10, M. Klinger11, M. Krajewska12, M. Kusztal13, M. Brzosko14, B. Iwona15, A. Dębska-Ślizień16, H. Storoniak17, B. Bułło-Piontecka18, W. Tłustochowicz19, J. Kur-Zalewska20, M. Wisłowska21, M. Madej22, A. Hawrot-Kawecka23, P. Głuszko24, M. Stasiek25, E. Kucharz26, J. Musiał27
- 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
- 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
- 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
- 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
- Department of Applied Mathematics, AGH University of Science and Technology, Kraków, Poland.
- Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland.
- Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland.
- Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Poland.
- Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Poland.
- Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Poland.
- Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Poland.
- Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Poland.
- Department of Nephrology and Transplantation Medicine, Wroclaw Medical University, Poland.
- Department of Rheumatology and Internal Diseases, Pomeranian Medical University in Szczecin, Poland.
- Department of Rheumatology and Internal Diseases, Pomeranian Medical University in Szczecin, Poland.
- Department of Nephrology, Transplantology and Internal Diseases, Medical University of Gdańsk, Poland.
- Department of Nephrology, Transplantology and Internal Diseases, Medical University of Gdańsk, Poland.
- Department of Nephrology, Transplantology and Internal Diseases, Medical University of Gdańsk, Poland.
- Department of Internal Medicine and Rheumatology, Military Medicine Institute, Warsaw, Poland.
- Department of Internal Medicine and Rheumatology, Military Medicine Institute, Warsaw, Poland.
- Department of Internal Diseases and Rheumatology, Central Clinical Hospital of the Ministry of the Interior and Administration, Warsaw, Poland.
- Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wrocław, Poland.
- Department of Internal Medicine and Metabolic Diseases, Medical University of Silesia, Katowice, Poland.
- Department of Rheumatology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
- Department of Rheumatology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.
- Department of Internal Medicine and Rheumatology, Medical University of Silesia, Katowice, Poland.
- 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland. jacek.musial@uj.edu.pl
CER13247
2021 Vol.39, N°2 ,Suppl.129
PI 0062, PF 0068
Diagnosis
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PMID: 32896241 [PubMed]
Received: 20/02/2020
Accepted : 08/06/2020
In Press: 01/09/2020
Published: 19/05/2021
Abstract
OBJECTIVES:
ANCA-associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown aetiology and the clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Thus, there is an unmet need for phenotype identification, especially among patients with granulomatosis with polyangiitis (GPA). Patients with microscopic polyangiitis (MPA) seem to be clinically much more uniform. Recently, three subcategories of AAV have been proposed and described as non-severe AAV, severe PR3-AAV, and severe MPO-AAV.
METHODS:
In line with these attempts, we decided to use an unbiased approach offered by latent class analysis (LCA) to subcategorise GPA and MPA in a large cohort of Polish AAV patients included in a multicentre POLVAS registry.
RESULTS:
LCA of our AAV group identified a four-class model of AAV, including previously proposed three subphenotypes and revealing a fourth (previously not described) clinically relevant subphenotype. This new subphenotype includes only GPA patients, usually diagnosed at a younger age as compared to other groups, and characterised by multiorgan involvement, high relapse rate, relatively high risk of death, but no end-stage kidney disease.
CONCLUSIONS:
Based on multiple clinical and serological variables, LCA methodology identified 4-class model of AAV. This newly described fourth class of AAV may be of clinical relevance and may require prompt diagnosis and aggressive treatment due to the multiorgan involvement, high risk of relapse and marked mortality among these relatively young GPA subjects.
