科研之友
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摘要
Rationale & objective: Phospholipase A(2) receptor (PLA(2)R)-associated membranous nephropathy (MN) with circulating serum autoantibodies to PLA(2)R (SAb(+)) but no deposits of PLA(2)R antigen in glomerular tissue by immunofluorescence (GAg(-)) has been reported. However, little is known about the clinicopathological characteristics or prognosis of this subtype of MN. Study design: Retrospective cohort study. Setting & participants: 130 SAb(+) patients in China with biopsy-proven MN who had follow-up data and received immunosuppressive therapy. The median follow-up was 16 (IQR, 9-25) months. Predictor: PLA(2)R antigen detection by immunofluorescence staining of kidney biopsy specimens. Outcomes: Complete remission (CR) was defined as proteinuria levels < 0.3 g/d and a > 50% decrease compared with a previously established baseline. Partial remission (PR) was defined as proteinuria levels < 3.5 g/d and a > 50% decrease compared with a previously established baseline. The kidney function outcome was defined as a > 40% decrease in estimated glomerular filtration rate (eGFR) at the end of the study compared with baseline. Analytical approach: Kaplan-Meier analysis of PR and CR comparing SAb(+)/GAg(+) and SAb(+)/GAg(-) patients. Cox proportional hazards models to examine these associations were adjusted for confounders. Results: Among 130 SAb(+) patients with PLA(2)R-associated MN, 18 were GAg(-). Compared with SAb(+)/GAg(+) patients, those who were SAb(+)/GAg(-) presented with more severe kidney injury as evidenced by higher SAb titer, greater proteinuria, lower serum albumin concentrations, lower eGFR (all P < 0.05), and more severe disease with higher chronicity scores (P < 0.001) on kidney biopsies. SAb(+)/GAg(-) patients exhibited a significantly lower probability of PR (P < 0.001) and CR (P = 0.03) and were more likely to experience a > 40% decrease in eGFR (P = 0.008) than patients who were SAb(+)/GAg(+). After adjusting for clinical and pathologic variables available at the time of biopsy, compared with SAb(+)/GAg(+) patients, SAb(+)/GAg(-) patients had a lower rate of experiencing remission (hazard ratio, 0.32 [95% CI, 0.15-0.68]; P = 0.003) and a higher rate of the > 40% eGFR decrease outcome (hazard ratio, 7.66 [95% CI, 1.54-38.08]; P = 0.01). Limitations: Retrospective study, small sample size, and lack of a uniform approach to treatment. Conclusions: Seropositive PLA(2)R-associated MN without PLA(2)R staining on kidney biopsy may represent a distinct clinical subtype with more severe disease and a worse prognosis. GAg(-) is independently associated with poor response to treatment and > 40% eGFR decrease in seropositive PLA(2)R-associated MN.
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单位南方医科大学
