Medizinische Universität Graz - Research portal
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Arnold, S; Mahrhold, J; Kerstein-Staehle, A; Riemekasten, G; Csernok, E; Hellmich, B; Venhoff, N; Thiel, J; Affeldt, K; Jahnke, A; Lamprecht, P.
Spectrum of ANCA-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study.
Clin Exp Rheumatol. 2023; 41(4):936-942
Doi: 10.55563/clinexprheumatol/299yme
Web of Science
PubMed
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- Co-authors Med Uni Graz
- Thiel Jens
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Humans - administration & dosage
- Antibodies, Antineutrophil Cytoplasmic - administration & dosage
- Retrospective Studies - administration & dosage
- Granulomatosis with Polyangiitis - diagnosis
- Churg-Strauss Syndrome - diagnosis
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - administration & dosage
- Myeloblastin - administration & dosage
- Peroxidase - administration & dosage
- Find related publications in this database (Keywords)
- eosinophilic granulomatosis with polyangiitis
- anti-neutrophil cytoplasmic antibodies
- myeloperoxidase
- pentraxin 3
- olfactomedin 4