Nika Kojc1, Alenka Bahovec1, Tanja Kersnik Levart2. 1. Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia. 2. Department of Nephrology, Division of Paediatrics, University Medical Centre, Ljubljana, Slovenia.
Abstract
AIM: To contribute additional clinical experience to the paucity of reports on C3 glomerulopathy (C3GP) in children, we are reporting our cohort of 11 children with C3GP, emphasizing the therapeutic options in this peculiar entity. METHODS: We describe the incidence, manifestation, histopathology findings, follow-up, treatment and outcome of C3GP in 11 children with C3GP by retrospectively analyzing their clinical charts and renal biopsy reports. RESULTS: Eleven C3GP patients were identified among 240 children who had undergone renal biopsy, accounting for a 4.6% incidence of C3GP. A light microscopy examination showed a membranoproliferative pattern (n = 8), mesangial proliferation (n = 1), a mesangial/membranoproliferative pattern (n = 1) and endocapillary proliferation (n = 1). All children presented with proteinuria of varying degrees, the majority of them with additional hematuria, three with full-blown nephrotic-nephritic syndrome, and two with renal insufficiency at presentation. Very diverse treatments were applied in our cohort of patients, from no specific treatment to different mono or combined anti-cellular immunosuppression treatments, as well as a trial of plasma therapy or eculizumab. Our results are in to some extend in concordance with other studies revealing that an optimal therapy for C3GP is still unknown, but we believe that a trial of classical immunosuppression before eculizumab is still worth trying, while eculizumab can have a beneficial effect, but not in all patients. CONCLUSION: A diverse histological pattern and clinical picture and no known optimal therapy are a hallmark of C3GP.
AIM: To contribute additional clinical experience to the paucity of reports on C3 glomerulopathy (C3GP) in children, we are reporting our cohort of 11 children with C3GP, emphasizing the therapeutic options in this peculiar entity. METHODS: We describe the incidence, manifestation, histopathology findings, follow-up, treatment and outcome of C3GP in 11 children with C3GP by retrospectively analyzing their clinical charts and renal biopsy reports. RESULTS: Eleven C3GP patients were identified among 240 children who had undergone renal biopsy, accounting for a 4.6% incidence of C3GP. A light microscopy examination showed a membranoproliferative pattern (n = 8), mesangial proliferation (n = 1), a mesangial/membranoproliferative pattern (n = 1) and endocapillary proliferation (n = 1). All children presented with proteinuria of varying degrees, the majority of them with additional hematuria, three with full-blown nephrotic-nephritic syndrome, and two with renal insufficiency at presentation. Very diverse treatments were applied in our cohort of patients, from no specific treatment to different mono or combined anti-cellular immunosuppression treatments, as well as a trial of plasma therapy or eculizumab. Our results are in to some extend in concordance with other studies revealing that an optimal therapy for C3GP is still unknown, but we believe that a trial of classical immunosuppression before eculizumab is still worth trying, while eculizumab can have a beneficial effect, but not in all patients. CONCLUSION: A diverse histological pattern and clinical picture and no known optimal therapy are a hallmark of C3GP.