C3-glomerulonephritis in New Zealand – a case series

Abstract

Background C3-glomerulonephritis can lead to progressive renal impairment from complement-mediated glomerular injury. Incidence and outcomes of C3-glomerulonephritis are not known in the New Zealand population. Methods We reviewed all cases of C3-glomerulonephritis from the past 10 years at a tertiary referral centre in New Zealand. Descriptive information on baseline characteristics and clinical outcomes was collected. Results Twenty-six patients were included (16 men; mean ± SD age 44 ± 25 years) with a median follow-up of 30 months. Disease incidence was 1.3 cases per million individuals, of which 42% were Pacific Islanders. Most patients presented with renal impairment, with a median (IQR) creatinine at diagnosis of 210 (146–300) μmol/L, and 11 (42%) patients presented with nephrotic syndrome. Seven (27%) patients progressed to end stage renal disease and 2 (8%) had died. End stage renal disease occurred in 20% of patients treated with immunosuppression and in 50% of those not treated. Complete remission was seen in 25% of patients treated with some form of immunosuppression and in 17% of those not treated. Conclusions Our results are consistent with previous descriptions of C3-glomerulonephritis. There was a suggestion of better clinical outcomes in patients treated with immunosuppression. There was a higher disease incidence in Pacific Islanders, which may indicate an underlying susceptibility to complement dysfunction in this population.

Can a Single Glomerulus Morphology Implicate Successful Therapy?

“…16 A case series of 26 cases in New Zealand with median follow-up to 30 months, combination therapy (MMF+ prednisolone) used in 12 cases, resulted in complete remission in 17% of cases, partial remission (stable disease) in 58% of cases, while, ESRD occurred in 50% of untreated cases, compared to 25% in treated cases. 17 Recurrence after kidney transplantation is high; up to 50% of recipients eventually lose their graft within 5 years of transplantation, a fact that requires patient and family counselling, and need for specific complement 5 (C5) blocking therapy as eculizumab to save kidney graft from progressive damage and loss. 18…”