Immunopathological analysis of the expression of glomerular exostosin 1 and exostosin 2 in Japanese patients with lupus nephritis

Glomerular Exostosin-Positivity is Associated With Disease Activity and Outcomes in Patients With Membranous Lupus Nephritis

“…in Japan proposed that apart from proteinuria level or renal function, the EXT1/EXT2 positivity is more associated with the concentration of serum autoantibodies (anti-dsDNA) and circulating immune complexes. 19 Indeed, our study showed a similar positive rate of serum lupus autoantibodies in EXT1/EXT2-positive and EXT1/EXT2-negative patients.…”

Analysis of the Expression of Exostosins and Clinicopathological Features in Membranous Lupus Nephritis in a Chinese Cohort

“…In addition, regarding the immunoserological markers representative of SLE disease activity, EXT1/EXT2-positive patients were more likely to have higher C3 level, higher proportion of less severe patients, and lower dsDNA titers than EXT1/EXT2-negative patients, similar to a previous Japanese study. 3 …”

“…Recently, the deposition of EXT1/EXT2 in LMN glomeruli have been validated in a few studies [2][3][4][5][6] . We confirmed that a subset of LMN patients was positive for EXT1/EXT2 in a Chinese cohort and the ratios of EXT-positive among combined and pure LMN were similar to studies by Ravindran et al and Saïdi et al 2,4 .…”

A Diagnostic and Therapeutic Dilemma Concerning Exostosin 1/Exostosin 2-associated Lupus-like Membranous Nephropathy with Positive Antinuclear Antibody in an Elderly Man with Various Immune Abnormalities

“…Wang et al reported that EXT1/EXT2-negative membranous LN patients were more prone to histologic transition than EXT1/EXT2-positive membranous LN patients (57% vs. 22%) after kidney relapse ( 23 ). Wada et al reported that Japanese membranous LN patients tended not to present with severe hypocomplementemia and elevated autoantibody titers compared to EXT1/2-negative proliferative LN patients ( 26 ). These findings suggest that EXT1/EXT2-positive and EXT1/EXT2-negative membranous LNs might be two distinct phenotypes and that EXT1/EXT2 may represent potential biomarkers, target antigens in secondary autoimmune MN or biomarkers for the development of subsequent autoimmune diseases, mainly SLE ( 1 , 3 ).…”