Are PR3 positive and MPO positive GPA the same disease?

Granulomatosis with Polyangiitis is a necrotising systemic vasculitis predominantly affecting small and medium sized vessels. It is predominantly associated with antibodies directed against proteinase 3, but a small number of individuals with this disease have antibodies directed against myeloperoxi...

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Bibliographic Details
Published in:International journal of rheumatic diseases 2019-01, Vol.22 (S1), p.86-89
Main Authors: Fordham, Sarah, Mukhtyar, Chetan
Format: Article
Language:English
Subjects:
Antineutrophil cytoplasmic antibodies
Autoantibodies - blood
Biomarkers - blood
Diagnosis, Differential
Disease
Genetic Predisposition to Disease
Genotype & phenotype
Granulomatosis
granulomatosis with polyangiitis
Granulomatosis with Polyangiitis - blood
Granulomatosis with Polyangiitis - diagnosis
Granulomatosis with Polyangiitis - genetics
Granulomatosis with Polyangiitis - immunology
Humans
Inflammatory diseases
Myeloblastin - immunology
myeloperoxidase
Peroxidase
Peroxidase - immunology
Phenotype
Phenotypes
Polymorphism, Single Nucleotide
Predictive Value of Tests
Prognosis
Proteinase
Proteinase 3
Risk Factors
Serotypes
Single-nucleotide polymorphism
Systemic vasculitis
Toll-Like Receptor 9 - genetics
Toll-Like Receptor 9 - immunology
vasculitides
Vein & artery diseases
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Description
Summary:Granulomatosis with Polyangiitis is a necrotising systemic vasculitis predominantly affecting small and medium sized vessels. It is predominantly associated with antibodies directed against proteinase 3, but a small number of individuals with this disease have antibodies directed against myeloperoxidase. The premise of this paper is to explore the possibility that these two serotypes maybe two separate diseases. There is evidence that the same single nucleotide polymorphisms in the TLR 9 gene is associated with increased risk for PR3 ANCA positivity but protects against MPO ANCA positivity. There is some evidence that MPO ANCA positive GPA disease may be ‘limited’ in its expression and be associated with the more indolent phenotype. The genotype may affect the serotype, and the serotype might affect the phenotype. There has been limited success in identifying how the difference in phenotype might affect outcomes. We are at a very early stage in our understanding of how these serotypes might be treated differently. This paper attempts to critically appraise the evidence available so far.
ISSN:1756-1841
1756-185X
DOI:10.1111/1756-185X.13278