TY - JOUR
TI - Occurrence and Antigenic Specificity of Perinuclear Anti-Neutrophil
Cytoplasmic Antibodies (P-ANCA) in Systemic Autoimmune Diseases
AU - Argyropoulou, Ourania D.
AU - Goules, V, Andreas
AU - Boutzios, Georgios
AU - and Tsirogianni, Alexandra
AU - Sfontouris, Charalampos
AU - Manoussakis,
AU - Menelaos N.
AU - Vlachoyiannopoulos, Panayiotis G.
AU - Tzioufas,
AU - Athanasios G.
AU - Kapsogeorgou, Efstathia K.
JO - Cell Stem Cell
PY - 2021
VL - 10
TODO - 8
SP - null
PB - MDPI
SN - 1934-5909
TODO - 10.3390/cells10082128
TODO - P-ANCA autoantibodies; myeloperoxidase; elastase; vasculitis; systemic
autoimmune rheumatic diseases
TODO - 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.
ER -