Akademik Veri Yönetim Sistemi
57th ESPN Annual Meeting, Athens, Yunanistan, 15 - 18 Ekim 2025, ss.13-14, (Özet Bildiri)
Aims/Purpose: This study aims to evaluate the clinical and pathological features, treatment approaches, treatment response, and complications in pediatric patients diagnosed with anti glomerular basement membrane (anti-GBM) disease. By compiling multinational data, we aim to better understand the disease course and inform future management. Methods: Data of patients under 18 years diagnosed with anti-GBM disease (based on the 2012 Chapel Hill Consensus) between 2003 and 2024 were retrospectively collected from multiple centers using a standardized form. Patients with ≥3 months of follow-up were included. Clinical, laboratory, biopsy, treatment, and outcome data were descriptively analyzed. Results: A total of 44 patients were included (female/male: 30/14) with a median age of 12 years (IQR 8.1–15.7). At presentation, the median serum creatinine level was 5.9 mg/dL (IQR 3–11.2), and median eGFR was 10 mL/min/1.73 m² (IQR 5.8–19.5). Pulmonary involvement was present in 50% of cases, primarily as pulmonary hemorrhage. Rapidly progressive glomerulonephritis (RPGN) was observed in 77% of patients. Anti-GBM antibodies were positive in 88.6%, and concomitant ANCA positivity was noted in 45%. Renal biopsy was performed in 40 patients; 32.5% showed >85% crescents on light microscopy, and linear IgG staining was observed in 92.5%. Apheresis was administered to 36 patients (median sessions: 10 [8–12]). IV steroid pulses were given to 42 patients (median: 3 [3–4.75]). IV cyclophosphamide was used in 32 patients (median: 3 doses [2–6]), and rituximab in 15 (median: 2 doses [2–4]). Maintenance therapy included mycophenolate mofetil in 15 patients and azathioprine in 7. The median follow-up duration was 26 months (IQR 5.6–46.9). At last follow-up, the median eGFR was 46.6 mL/min/1.73 m² (IQR 8.8–68.6). A total of 19 patients developed end-stage kidney disease; 6 underwent kidney transplantation, 13 were on chronic hemodialysis, and 6 were receiving peritoneal dialysis. Four deaths were reported—one due to pulmonary complications and three due to cerebrovascular events. Conclusion: This international cohort is one of the largest pediatric anti-GBM series to date. The f indings reflect the disease’s severity and need for timely, intensive management. Further analyses are ongoing to identify prognostic markers and improve outcomes.