Vasculitis & polymyalgia rheumatica
Spectrum of anti-neutrophil cytoplasmic antibody (ANCA)-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study
Authors' conclusion
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.
Study context
- Population
- Eosinophilic granulomatosis with polyangiitis (EGPA) patients from three German tertiary referral centres for vasculitis
- Participants
- 73 patients
- Exposure
- Patient characteristics and clinical manifestations compared by ANCA status
- MPO-ANCA positiven = 8
- PTX3-ANCA positiven = 5
- PR3-ANCA positiven = 2
- OLM4-ANCA positiven = 2
- Outcome
- Spectrum of ANCA antigen-specificities in EGPA
Methodological detailsmulticentre, three German tertiary referral centres, PTX3- and OLM4-ANCA determined with a prototype cell-based research assay, in addition to in-house ANCA testing
- Multicentre
- Three German tertiary referral centres
- PTX3- and OLM4-ANCA determined with a prototype cell-based research assay, in addition to in-house ANCA testing
Main finding
As reported in the abstract
ANCA antigen-specificity prevalence
- One PR3-ANCA positive patient also positive for BPI-ANCA
- 1
Observational data: associations, not effects.
Further findings
Organ involvement by MPO-ANCA status
- MPO-ANCA positive: significantly more frequent peripheral nervous system (PNS) and pulmonary involvement and less frequent heart involvement vs MPO-ANCA negative
Organ involvement by PTX3-ANCA status
- PTX3-ANCA positive: significantly higher prevalence of ENT, pulmonary, gastrointestinal and PNS involvement and lower prevalence of renal and CNS involvement vs PTX3-ANCA negative
Organ involvement in PR3-ANCA and OLM4-ANCA positive patients
- Multiorgan involvement
Original publication
Spectrum of ANCA-specificities in eosinophilic granulomatosis with polyangiitis. A retrospective multicentre study. Clin Exp Rheumatol. Published 28 October 2022. doi:10.
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