ESPN 51th Annual Meeting

ESPN 2018


 
CLINICAL CHARACTERISTICS AND PROGNOSIS OF PATIENTS WITH C3 GLOMERULOPATHY: A SINGLE CENTRE EXPERIENCE
IBRAHIM GOKCE 1 NESLIHAN CICEK 1 MEHTAP SAK 1 NURDAN YILDIZ 1 HANDAN KAYA 2 HARIKA ALPAY 1

1- MARMARA UNIVERSITY MEDICAL SCHOOL, DEPARTMENT OF PEDIATRIC NEPHROLOGY, ISTANBUL, TURKEY
2- MARMARA UNIVERSITY MEDICAL SCHOOL, DEPARTMENT OF PATHOLOGY, ISTANBUL, TURKEY
 
Introduction:

 

C3 glomerulopathy(C3G) is a recently identified disease caused by dysregulation of the alternative complement pathway which is characterized by  isolated or dominant deposits of C3 on immunofluorescence. The differential diagnosis of two variants of C3G, dense deposit disease(DDD) and C3 glomerulonephritis(C3GN) is based solely on electron microscopic features.

Material and methods:

 

We analyzed the clinical characteristics, pathologic findings, treatments and long-term renal outcomes of 6 patients with C3G followed in our pediatric nephrology department retrospectively. We evaluated estimated glomerular filtration rate(eGFR), urinary protein excretion, serum creatinine, albumin levels, genetic tests and treatments of the patients.

Results:

 

Three patients(50%) were female and 3(50%) were male. The mean age at presentation, at diagnosis and the mean follow up time were 16.99±4.56(8-20.8), 9.82±7.38(1-18.4) and 7±6.57(0.1-18.2) years respectively. The mean urinary protein excretion was 50.66±25.42(23-83) mg/m2/h, the mean serum creatinine  and albumin levels were  1.29±1.31(0.48-3.8) mg/dl and 3.65±0.27(3.3-3.9) gr/dl respectively. Serum C3 levels were low in all patients. The genetic test for factor H was  performed for 4 patients, homozygous mutation was found in 2 patients, heterozygous in 1 patient and no mutation in 1 patient. One patient progressed to end stage renal disease(ESRD) and has been treated with hemodialysis for the  last 3 months, another patient is followed as chronic kidney disease stage 3 with eGFR 43.8 ml/min/1.73m2 whereas eGFR is still normal in the rest of patients. Corticosteroids, cyclophosphamide, cyclosporine A, MMF and rituximab were used for the treatment of patients. Two patients with homozygous mutation who were resistant to all therapies were treated with eculizumab.

Conclusions:

 

The long-term prognosis of C3G is generally unfavorable and it may progress to ESRD. The investigations of genetic mutations of alternative complement pathway is important in these patients and eculizumab, inhibitor of C5a, is a favorable treatment in patients resistant to other therapies.