@article{3030469,
    title = "Occurrence and Antigenic Specificity of Perinuclear Anti-Neutrophil
Cytoplasmic Antibodies (P-ANCA) in Systemic Autoimmune Diseases",
    author = "Argyropoulou, Ourania D. and Goules, V, Andreas and Boutzios, Georgios and and Tsirogianni, Alexandra and Sfontouris, Charalampos and Manoussakis, and Menelaos N. and Vlachoyiannopoulos, Panayiotis G. and Tzioufas, and Athanasios G. and Kapsogeorgou, Efstathia K.",
    journal = "Cell Stem Cell",
    year = "2021",
    volume = "10",
    number = "8",
    publisher = "MDPI",
    issn = "1934-5909",
    doi = "10.3390/cells10082128",
    keywords = "P-ANCA autoantibodies; myeloperoxidase; elastase; vasculitis; systemic
autoimmune rheumatic diseases",
    abstract = "Perinuclear anti-neutrophilic cytoplasmic antibodies (P-ANCA) recognize
heterogeneous antigens, including myeloperoxidase (MPO), lactoferrin,
elastase, cathepsin-G and bactericidal/permeability-increasing protein.
Although P-ANCA have diagnostic utility in vasculitides, they may also
be found in patients with various other systemic autoimmune rheumatic
diseases (SARDs). Nevertheless, the clinical significance and the
targets recognized by P-ANCA in such patients remain unclear. For this
purpose, herein we investigated the occurrence of ANCA-related antigenic
specificities in 82 P-ANCA-positive sera by multiplex ELISA, as well as
their association with other autoantibodies. The P-ANCA-positive sera
corresponded to patients with vasculitides (n = 24), systemic lupus
erythematosus (n = 28), antiphospholipid syndrome (n = 5), Sjogren’s
syndrome (n = 7), rheumatoid arthritis (n = 3), systemic scleroderma (n
= 1), sarcoidosis (n = 1) and Hashimoto ‘ s thyroiditis (n = 13). In
most P-ANCA-positive patients studied (51/82, 62.3%), these
autoantibodies occurred in high titers (>1:160). The analysis of
P-ANCA-positive sera revealed reactivity to MPO in only 50% of patients
with vasculitides, whereas it was infrequent in the other disease groups
studied. Reactivity to other P-ANCA-related autoantigens was also rarely
detected. Our findings support that high P-ANCA titers occur in SARD.
The P-ANCA-positive staining pattern is associated with MPO specificity
in vasculitides, while in other autoimmune diseases, it mostly involves
unknown autoantigens."
}