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Spectrum of ANCA-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study
S. Arnold1, J. Mahrhold2, A. Kerstein-Staehle3, G. Riemekasten4, E. Csernok5, B. Hellmich6, N. Venhoff7, J. Thiel8, K. Affeldt9, A. Jahnke10, P. Lamprecht11
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany. sabrina.arnold@uksh.de
- Department of Internal Medicine, Rheumatology and Immunology, Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Kirchheim unter Teck, Germany.
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
- Department of Internal Medicine, Rheumatology and Immunology, Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Kirchheim unter Teck, Germany.
- Department of Internal Medicine, Rheumatology and Immunology, Vasculitis Centre South, Medius Kliniken, Teaching Hospital University of Tübingen, Kirchheim unter Teck, Germany.
- Department of Rheumatology and Clinical Immunology, Medical Center, University of Freiburg, Germany.
- Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, Medical University Graz, Austria, and Department of Rheumatology and Clinical Immunology, Medical Center, University of Freiburg, Germany.
- Institute of Experimental Immunology affiliated to EUROIMMUN AG, Lübeck, Germany.
- Institute of Experimental Immunology affiliated to EUROIMMUN AG, Lübeck, Germany.
- Department of Rheumatology and Clinical Immunology, University of Lübeck, Germany.
CER16317
2023 Vol.41, N°4
PI 0936, PF 0942
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PMID: 37073637 [PubMed]
Received: 28/10/2022
Accepted : 13/03/2023
In Press: 05/04/2023
Published: 18/04/2023
Abstract
OBJECTIVES:
To determine the spectrum of anti-neutrophil cytoplasmic antibody (ANCA) antigen-specificities in eosinophilic granulomatosis with polyangiitis (EGPA), an ANCA-associated vasculitis (AAV) entity.
METHODS:
We conducted a retrospective analysis of 73 EGPA patients from three German tertiary referral centres for vasculitis. In addition to in-house ANCA testing, pentraxin 3 (PTX3)- and olfactomedin 4 (OLM4)-ANCA were determined using a prototype cell-based assay for research (EUROIMMUN, Lübeck, Germany). Patient characteristics and clinical manifestations were evaluated and compared based on ANCA status.
RESULTS:
Myeloperoxidase (MPO)-ANCA positive patients (n=8; 11%) significantly more frequently displayed peripheral nervous system (PNS) and pulmonary involvement and less frequently heart involvement compared to MPO-ANCA negative patients. PTX3-ANCA positive patients (n=5; 6.8%) had a significantly higher prevalence of ear, nose and throat, pulmonary, gastrointestinal and PNS involvement, and a lower prevalence of renal and central nervous system involvement compared to PTX3-ANCA negative patients. Proteinase 3 (PR3)-ANCA and OLM4-ANCA were detected in 2 patients (2.7%) each with multiorgan involvement. One PR3-ANCA positive patient was also positive for bactericidal permeability increasing protein (BPI)-ANCA.
CONCLUSIONS:
In addition to MPO, the spectrum of ANCA antigen specificities includes various other target antigens such as PR3, BPI, PTX3, and OLM4, potentially segregating further EGPA subgroups. A lower prevalence of MPO-ANCA was detected in this study compared with other studies. OLM4 is reported as novel ANCA antigen-specificity in EGPA, and thus AAV.
