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16-01-2024 | Hematuria | Original Article

Childhood-onset IgA nephropathy: is long-term recovery possible?

Authors: Luca Antonucci, Laura Fuiano, Antonio Gargiulo, Alessandra Gianviti, Andrea Onetti Muda, Francesca Diomedi Camassei, Marina Vivarelli, Francesco Emma

Published in: Pediatric Nephrology | Issue 6/2024

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Abstract

Background

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide. While studies have primarily focused on identifying risk factors for disease progression, very few data exist on the likelihood of achieving complete recovery from the disease.

Methods

We conducted a single-center retrospective study on all consecutive patients with biopsy-proven IgAN diagnosed between 1986 and 2018 in our pediatric center. Biopsies were classified according to the MEST-C Oxford classification score. “Complete clinical remission” was defined as the absence of proteinuria, hematuria, and hypertension in patients with normal kidney function who had been off therapy for more than 2 years.

Results

Overall, 153 patients with age at onset of 10.6 ± 4 years were enrolled in the study. Of these, 41 achieved “complete clinical remission.” The estimated probability of complete clinical remission at 10 years was 43% (95%CI 33–54). However, seven patients relapsed within 10 years. Multivariable analysis showed that higher age at onset (HR 0.89, 95%CI 0.80–0.98, p = 0.017) and segmental glomerulosclerosis lesions (HR 0.28, 95%CI 0.10–0.79, p = 0.017) decreased significantly the chances of achieving complete clinical remission. Immunosuppressive therapy was not significantly associated with clinical outcomes.

Conclusions

Approximately one-third of patients with pediatric-onset IgAN achieve prolonged remission, in particular, very young children at disease onset without sclerotic glomerular lesions. Longer term follow-up is needed to assess if these patients have achieved permanent remission.

Graphical abstract

Appendix
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Literature
1.
go back to reference D’Amico G (1987) The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med 64:709–727PubMed D’Amico G (1987) The commonest glomerulonephritis in the world: IgA nephropathy. Q J Med 64:709–727PubMed
9.
go back to reference Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D’Agati V, D’Amico G, Emancipator S, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Leung CB, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int 76:534–545. https://doi.org/10.1038/ki.2009.243CrossRefPubMed Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D’Agati V, D’Amico G, Emancipator S, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Leung CB, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int 76:534–545. https://​doi.​org/​10.​1038/​ki.​2009.​243CrossRefPubMed
11.
go back to reference Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263CrossRefPubMed Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263CrossRefPubMed
14.
go back to reference Lurbe E, Agabiti-Rosei E, Cruickshank JK, Dominiczak A, Erdine S, Hirth A, Invitti C, Litwin M, Mancia G, Pall D, Rascher W, Redon J, Schaefer F, Seeman T, Sinha M, Stabouli S, Webb NJ, Wühl E, Zanchetti A (2016) 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 34:1887–1920. https://doi.org/10.1097/hjh.0000000000001039CrossRefPubMed Lurbe E, Agabiti-Rosei E, Cruickshank JK, Dominiczak A, Erdine S, Hirth A, Invitti C, Litwin M, Mancia G, Pall D, Rascher W, Redon J, Schaefer F, Seeman T, Sinha M, Stabouli S, Webb NJ, Wühl E, Zanchetti A (2016) 2016 European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 34:1887–1920. https://​doi.​org/​10.​1097/​hjh.​0000000000001039​CrossRefPubMed
15.
go back to reference Levin A, Stevens PE, Bilous RW, Coresh J, De Francisco ALM, De Jong PE, Griffith KE, Hemmelgarn BR, Iseki K, Lamb EJ, Levey AS, Riella MC, Shlipak MG, Wang H, White CT, Winearls CG (2013) Kidney disease: improving global outcomes (KDIGO) CKD work group. KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl 3:1–150 Levin A, Stevens PE, Bilous RW, Coresh J, De Francisco ALM, De Jong PE, Griffith KE, Hemmelgarn BR, Iseki K, Lamb EJ, Levey AS, Riella MC, Shlipak MG, Wang H, White CT, Winearls CG (2013) Kidney disease: improving global outcomes (KDIGO) CKD work group. KDIGO 2012 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl 3:1–150
18.
go back to reference Coppo R, Troyanov S, Camilla R, Hogg RJ, Cattran DC, Cook HT, Feehally J, Roberts IS, Amore A, Alpers CE, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D’Agati V, D’Amico G, Emancipator SN, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo AB, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Li LS, Li PK, Liu ZH, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2010) The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int 77:921–927. https://doi.org/10.1038/ki.2010.43CrossRefPubMed Coppo R, Troyanov S, Camilla R, Hogg RJ, Cattran DC, Cook HT, Feehally J, Roberts IS, Amore A, Alpers CE, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D’Agati V, D’Amico G, Emancipator SN, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo AB, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Li LS, Li PK, Liu ZH, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2010) The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int 77:921–927. https://​doi.​org/​10.​1038/​ki.​2010.​43CrossRefPubMed
20.
go back to reference Shima Y, Nakanishi K, Kamei K, Togawa H, Nozu K, Tanaka R, Sasaki S, Iijima K, Yoshikawa N (2011) Disappearance of glomerular IgA deposits in childhood IgA nephropathy showing diffuse mesangial proliferation after 2 years of combination/prednisolone therapy. Nephrol Dial Transplant 26:163–169. https://doi.org/10.1093/ndt/gfq387CrossRefPubMed Shima Y, Nakanishi K, Kamei K, Togawa H, Nozu K, Tanaka R, Sasaki S, Iijima K, Yoshikawa N (2011) Disappearance of glomerular IgA deposits in childhood IgA nephropathy showing diffuse mesangial proliferation after 2 years of combination/prednisolone therapy. Nephrol Dial Transplant 26:163–169. https://​doi.​org/​10.​1093/​ndt/​gfq387CrossRefPubMed
21.
go back to reference Suh JS, Jang KM, Hyun H, Cho MH, Lee JH, Park YS, Oh JH, Kim JH, Yoo KH, Chung WY, Kim SH, Kim K, Lee DY, Lee JW, Cho MH, Park H, Koo JW, Han KH, Yang EM, Lee KH, Shin JI, Cho H, Kim KS, Ha IS, Park YH, Kang HG (2020) Remission of proteinuria may protect against progression to chronic kidney disease in pediatric-onset IgA nephropathy. J Clin Med 9. https://doi.org/10.3390/jcm9072058 Suh JS, Jang KM, Hyun H, Cho MH, Lee JH, Park YS, Oh JH, Kim JH, Yoo KH, Chung WY, Kim SH, Kim K, Lee DY, Lee JW, Cho MH, Park H, Koo JW, Han KH, Yang EM, Lee KH, Shin JI, Cho H, Kim KS, Ha IS, Park YH, Kang HG (2020) Remission of proteinuria may protect against progression to chronic kidney disease in pediatric-onset IgA nephropathy. J Clin Med 9. https://​doi.​org/​10.​3390/​jcm9072058
22.
go back to reference 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, H KS, 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 (2017) Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort. Pediatr Nephrol 32:139–150. https://doi.org/10.1007/s00467-016-3469-3CrossRefPubMed 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, H KS, 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 (2017) Risk factors for progression in children and young adults with IgA nephropathy: an analysis of 261 cases from the VALIGA European cohort. Pediatr Nephrol 32:139–150. https://​doi.​org/​10.​1007/​s00467-016-3469-3CrossRefPubMed
26.
31.
go back to reference Coppo R, D’Arrigo G, Tripepi G, Russo ML, Roberts ISD, Bellur S, Cattran D, Cook TH, Feehally J, Tesar V, Maixnerova D, Peruzzi L, Amore A, Lundberg S, Di Palma AM, Gesualdo L, Emma F, Rollino C, Praga M, Biancone L, Pani A, Feriozzi S, Polci R, Barratt J, Del Vecchio L, Locatelli F, Pierucci A, Caliskan Y, Perkowska-Ptasinska A, Durlik M, Moggia E, Ballarin JC, Wetzels JFM, Goumenos D, Papasotiriou M, Galesic K, Toric L, Papagianni A, Stangou M, Benozzi L, Cusinato S, Berg U, Topaloglu R, Maggio M, Ots-Rosenberg M, D’Amico M, Geddes C, Balafa O, Quaglia M, Cravero R, Lino Cirami C, Fellstrom B, Floege J, Egido J, Mallamaci F, Zoccali C (2020) Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford Classification for IgA Nephropathy (VALIGA) update. Nephrol Dial Transplant 35:1002–1009. https://doi.org/10.1093/ndt/gfy302CrossRefPubMed Coppo R, D’Arrigo G, Tripepi G, Russo ML, Roberts ISD, Bellur S, Cattran D, Cook TH, Feehally J, Tesar V, Maixnerova D, Peruzzi L, Amore A, Lundberg S, Di Palma AM, Gesualdo L, Emma F, Rollino C, Praga M, Biancone L, Pani A, Feriozzi S, Polci R, Barratt J, Del Vecchio L, Locatelli F, Pierucci A, Caliskan Y, Perkowska-Ptasinska A, Durlik M, Moggia E, Ballarin JC, Wetzels JFM, Goumenos D, Papasotiriou M, Galesic K, Toric L, Papagianni A, Stangou M, Benozzi L, Cusinato S, Berg U, Topaloglu R, Maggio M, Ots-Rosenberg M, D’Amico M, Geddes C, Balafa O, Quaglia M, Cravero R, Lino Cirami C, Fellstrom B, Floege J, Egido J, Mallamaci F, Zoccali C (2020) Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford Classification for IgA Nephropathy (VALIGA) update. Nephrol Dial Transplant 35:1002–1009. https://​doi.​org/​10.​1093/​ndt/​gfy302CrossRefPubMed
Metadata
Title
Childhood-onset IgA nephropathy: is long-term recovery possible?
Authors
Luca Antonucci
Laura Fuiano
Antonio Gargiulo
Alessandra Gianviti
Andrea Onetti Muda
Francesca Diomedi Camassei
Marina Vivarelli
Francesco Emma
Publication date
16-01-2024
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 6/2024
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-023-06259-4

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