Spectrum of ANCA-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study.

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.

• Authors: Sabrina Arnold, Juliane Mahrhold, Anja Kerstein-Staehle, Gabriela Riemekasten, Elena Csernok, Bernhard Hellmich, Nils Venhoff, Jens Thiel, Kai Affeldt, Annika Jahnke, Peter Lamprecht
• Journal: Clinical and experimental rheumatology (Clin Exp Rheumatol) Vol. 41 Issue 4 Pg. 936-942 (Apr 2023) ISSN: 0392-856X [Print] Italy
• PMID: 37073637 (Publication Type: Multicenter Study, Journal Article)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Myeloblastin
• Peroxidase

Topics

• Humans
• Antibodies, Antineutrophil Cytoplasmic
• Retrospective Studies
• Granulomatosis with Polyangiitis (diagnosis)
• Churg-Strauss Syndrome (diagnosis)
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Myeloblastin
• Peroxidase