ESPN 50th Annual Meeting

ESPN 2017


 
ANCA-associated vasculitis with renal involvement in children: clinical characteristic and outcome
INA KAZYRA 1 ELENA KILCHEVSKAJA 2 MOHSEN HOSHAYARIKHANI 1 Farida FOTOVATKASBSHIRAZI 1

1- BELARUS STATE MEDICAL UNIVERSITY
2- 2ND CHILDRENS HOSPITAL
 
Introduction:

 ANCA-associated glomerulonephritis(GN) is a rare disorder in children, presentation and outcomes vary significantly among patients. Renal involvement contributes significantly to the morbidity with high numbers of patients progressing to end-stage kidney disease.  

Material and methods:

 We review 5 patients (2boys, 6-17yrs, mediana 8.0) with ANCA-positive GN from Belarus National Center for pediatric nephrology patients. Caspase-1, TNFα, IL1β, BAFF, RANTES, VEGF, TGF1β, IgG ANCA (PR3, MPO, BPI, elastase, lysozyme, lactoferrin, cathepsinG), 24 hours monitoring of blood pressure(BP), ECHO-CG, carotid intima media thickness(cIMT), left ventricles mass index (LVMI), relative thickness of left ventricles wall (RTLV), body mass index (BMI), serum lipids, glucose, uric acide levels, eGFR were measured. 

Results:

 The median time from presentation until first clinical or serologic signs was 12months (6-29mo). The main organs involved were: renal in 5(100%); respiratory tract 5 (100%); joints 2(40%); skin rash 3(60%); eyes 2(40%). PR3 and MPO-ANCA, caspase-1, TNFα, IL1β, BAFF, RANTES, VEGF, TGF1β levels were positively correlated with disease activity and organ involvement. Hypertension was observed in 100%, dilatations of LV in 40%, reduced ejection fraction in 20% of patients. LVMI, RTLV, BMI and cIMT were higher compared with healthy(p<0.05). The mean glucose, uric acid and cholesterol level was significantly higher than in healthy(p<0.05), lipoproteins of low and very low density prevailed.

Conclusions:

 1 patient was treated with plasmapheresis and prednisolone pulses at presentation, required dialysis. He died after 12months from the manifestation of the disease due to infection. Induction therapy in others included cyclophosphamide pulses and steroids, maintenance azathioprine and steroids. Cardiovascular, metabolic and immune abnormalities were correlated with disease activity and duration. Such patients are at high risk of early development of cardiovascular complications requiring early correction.