부산대학교 도서관

BACKGROUND: Autoantibodies are common in glomerulonephritis, but the clinical benefit of rapid elimination is not determined, even in anti-glomerular basement membrane (GBM) disease. Even less is known about the importance of autoantibody characteristics, including epitope specificity and IgG subclass distribution. By utilizing samples from the GOOD-IDES-01 trial, where imlifidase, cleaving all IgG in vivo within hours, was given to 15 anti-GBM patients, we aimed to address this by characterizing the autoantibody profile in anti-GBM patients.METHODS: In the GOOD-IDES-01 trial, plasmapheresis was (re)started if anti-GBM antibodies rebounded. Serum samples were collected prospectively for 6 months and analyzed for anti-GBM epitope specificity using recombinant constructs of the EA and EB epitopes, IgG subclass using monoclonal antibodies, and anti-neutrophil cytoplasmic antibodies (ANCA). The results were correlated with clinical data.RESULTS: Patients with a rebound (n = 10) tended to have lower eGFR at 6 months (11 vs 34 mL/min/1.73m2, p = 0.055), and patients with dialysis at 6 months had a higher EB/EA ratio at rebound (0.8 vs 0.5, p = 0.047). Moreover, 2 patients demonstrated increasing epitope restriction and several patients displayed a shift in subclass distribution at rebound. Six patients were double positive for ANCA. ANCA rebound was seen in 50% of patients, only 1 patient remained ANCA positive at 6 months.CONCLUSIONS: In this study, rebound of anti-GBM antibodies, especially if directed against the EB epitope, was associated with a worse outcome. This supports the notion that all means should be used to eliminate anti-GBM antibodies. ANCA was in this study removed early and long-term by imlifidase and cyclophosphamide.