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. 2017 Jan;32(1):139-150.
doi: 10.1007/s00467-016-3469-3. Epub 2016 Aug 25.

Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort

Rosanna Coppo  1 , Danilo Lofaro  2 , Roberta R Camilla  3 , Shubha Bellur  4 , Daniel Cattran  5 , H Terence Cook  6 , Ian S D Roberts  4 , Licia Peruzzi  3 , Alessandro Amore  3 , Francesco Emma  7 , Laura Fuiano  7 , Ulla Berg  8 , Rezan Topaloglu  9 , Yelda Bilginer  9 , Loreto Gesualdo  10 , Rosaria Polci  11 , Malgorzata Mizerska-Wasiak  12 , Yasar Caliskan  13 , Sigrid Lundberg  14 , Giovanni Cancarini  15 , Colin Geddes  16 , Jack Wetzels  17 , Andrzej Wiecek  18 , Magdalena Durlik  19 , Stefano Cusinato  20 , Cristiana Rollino  21 , Milena Maggio  22 , Manuel Praga  23 , Hilde K Smerud  24 , Vladimir Tesar  25 , Dita Maixnerova  25 , Jonathan Barratt  26 , Teresa Papalia  2 , Renzo Bonofiglio  2 , Gianna Mazzucco  27 , Costantinos Giannakakis  28 , Magnus Soderberg  8 , Diclehan Orhan  9 , Anna Maria Di Palma  10 , Jadwiga Maldyk  12 , Yasemin Ozluk  13 , Birgitta Sudelin  14 , Regina Tardanico  15 , David Kipgen  16 , Eric Steenbergen  17 , Henryk Karkoszka  18 , Agnieszka Perkowska-Ptasinska  19 , Franco Ferrario  29 , Eduardo Gutierrez  23 , Eva Honsova  25
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Free article

Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort

Rosanna Coppo et al. Pediatr Nephrol. 2017 Jan.
Free article

Erratum in

  • Erratum to: Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort.
    Coppo R, Lofaro D, Camilla RR, Bellur S, Cattran D, Cook HT, Roberts IS, Peruzzi L, Amore A, Emma F, Fuiano L, Berg U, Topaloglu R, Bilginer Y, Gesualdo L, Polci R, Mizerska-Wasiak M, Caliskan Y, Lundberg S, Cancarini G, Geddes C, Wetzels J, Wiecek A, Durlik M, Cusinato S, Rollino C, Maggio M, Praga M, Smerud HK, Tesar V, Maixnerova D, Barratt J, Papalia T, Bonofiglio R, Mazzucco G, Giannakakis C, Soderberg M, Orhan D, Di Palma AM, Maldyk J, Ozluk Y, Sudelin B, Tardanico R, Kipgen D, Steenbergen E, Karkoszka H, Perkowska-Ptasinska A, Ferrario F, Gutierrez E, Honsova E. Coppo R, et al. Pediatr Nephrol. 2017 Jan;32(1):193-194. doi: 10.1007/s00467-016-3506-2. Pediatr Nephrol. 2017. PMID: 27704253 No abstract available.

Abstract

Background: There is a need for early identification of children with immunoglobulin A nephropathy (IgAN) at risk of progression of kidney disease.

Methods: Data on 261 young patients [age <23 years; mean follow-up of 4.9 (range 2.5-8.1) years] enrolled in VALIGA, a study designed to validate the Oxford Classification of IgAN, were assessed. Renal biopsies were scored for the presence of mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis (T1-2) (MEST score) and crescents (C1). Progression was assessed as end stage renal disease and/or a 50 % loss of estimated glomerular filtration rate (eGFR) (combined endpoint) as well as the rate of renal function decline (slope of eGFR). Cox regression and tree classification binary models were used and compared.

Results: In this cohort of 261 subjects aged <23 years, Cox analysis validated the MEST M, S and T scores for predicting survival to the combined endpoint but failed to prove that these scores had predictive value in the sub-group of 174 children aged <18 years. The regression tree classification indicated that patients with M1 were at risk of developing higher time-averaged proteinuria (p < 0.0001) and the combined endpoint (p < 0.001). An initial proteinuria of ≥0.4 g/day/1.73 m2 and an eGFR of <90 ml/min/1.73 m2 were determined to be risk factors in subjects with M0. Children aged <16 years with M0 and well-preserved eGFR (>90 ml/min/1.73 m2) at presentation had a significantly high probability of proteinuria remission during follow-up and a higher remission rate following treatment with corticosteroid and/or immunosuppressive therapy.

Conclusion: This new statistical approach has identified clinical and histological risk factors associated with outcome in children and young adults with IgAN.

Keywords: IgA nephropathy; Pathology classification; Progression; Proteinuria; Risk factors.

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