Outcome of childhood-onset full-house nephropathy

Barbara Ruggiero, Marina Vivarelli, Alessandra Gianviti, Carmine Pecoraro, R. Licia Peruzzi, Elisa Benetti, Giovanna Ventura, Marco Pennesi, Luisa Murer, Rosanna Coppo, Francesco Emma

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.

METHODS: Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria ≤50% from baseline), relapse (estimated glomerular filtration rate <25% and/or proteinuria ≥50% from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.

RESULTS: Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9%) achieved partial and 32 (76.2%) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8%. Of those achieving remission, 18% had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.

CONCLUSIONS: The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.

Original languageEnglish
JournalNephrology Dialysis Transplantation
DOIs
Publication statusE-pub ahead of print - Jun 7 2016

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Chronic Renal Insufficiency
Proteinuria
Recurrence
Kidney
Lupus Nephritis
Rheumatology
Glomerular Filtration Rate
Proportional Hazards Models
Immunosuppression
Age Groups
Regression Analysis
Maintenance
Biopsy
Therapeutics

Keywords

  • Journal Article

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Outcome of childhood-onset full-house nephropathy. / Ruggiero, Barbara; Vivarelli, Marina; Gianviti, Alessandra; Pecoraro, Carmine; Peruzzi, R. Licia; Benetti, Elisa; Ventura, Giovanna; Pennesi, Marco; Murer, Luisa; Coppo, Rosanna; Emma, Francesco.

In: Nephrology Dialysis Transplantation, 07.06.2016.

Research output: Contribution to journalArticle

Ruggiero, B, Vivarelli, M, Gianviti, A, Pecoraro, C, Peruzzi, RL, Benetti, E, Ventura, G, Pennesi, M, Murer, L, Coppo, R & Emma, F 2016, 'Outcome of childhood-onset full-house nephropathy', Nephrology Dialysis Transplantation. https://doi.org/10.1093/ndt/gfw230
Ruggiero, Barbara ; Vivarelli, Marina ; Gianviti, Alessandra ; Pecoraro, Carmine ; Peruzzi, R. Licia ; Benetti, Elisa ; Ventura, Giovanna ; Pennesi, Marco ; Murer, Luisa ; Coppo, Rosanna ; Emma, Francesco. / Outcome of childhood-onset full-house nephropathy. In: Nephrology Dialysis Transplantation. 2016.
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abstract = "BACKGROUND: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.METHODS: Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria ≤50{\%} from baseline), relapse (estimated glomerular filtration rate <25{\%} and/or proteinuria ≥50{\%} from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.RESULTS: Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9{\%}) achieved partial and 32 (76.2{\%}) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8{\%}. Of those achieving remission, 18{\%} had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.CONCLUSIONS: The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.",
keywords = "Journal Article",
author = "Barbara Ruggiero and Marina Vivarelli and Alessandra Gianviti and Carmine Pecoraro and Peruzzi, {R. Licia} and Elisa Benetti and Giovanna Ventura and Marco Pennesi and Luisa Murer and Rosanna Coppo and Francesco Emma",
note = "{\circledC} The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.",
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TY - JOUR

T1 - Outcome of childhood-onset full-house nephropathy

AU - Ruggiero, Barbara

AU - Vivarelli, Marina

AU - Gianviti, Alessandra

AU - Pecoraro, Carmine

AU - Peruzzi, R. Licia

AU - Benetti, Elisa

AU - Ventura, Giovanna

AU - Pennesi, Marco

AU - Murer, Luisa

AU - Coppo, Rosanna

AU - Emma, Francesco

N1 - © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

PY - 2016/6/7

Y1 - 2016/6/7

N2 - BACKGROUND: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.METHODS: Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria ≤50% from baseline), relapse (estimated glomerular filtration rate <25% and/or proteinuria ≥50% from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.RESULTS: Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9%) achieved partial and 32 (76.2%) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8%. Of those achieving remission, 18% had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.CONCLUSIONS: The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.

AB - BACKGROUND: Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.METHODS: Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria ≤50% from baseline), relapse (estimated glomerular filtration rate <25% and/or proteinuria ≥50% from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.RESULTS: Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9%) achieved partial and 32 (76.2%) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8%. Of those achieving remission, 18% had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.CONCLUSIONS: The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.

KW - Journal Article

U2 - 10.1093/ndt/gfw230

DO - 10.1093/ndt/gfw230

M3 - Article

C2 - 27270291

JO - Nephrology Dialysis Transplantation

JF - Nephrology Dialysis Transplantation

SN - 0931-0509

ER -