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Outcome of childhood-onset full-house nephropathy
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...
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| Published in: | Nephrology, dialysis, transplantation dialysis, transplantation, 2017-07, Vol.32 (7), p.1194-1204 |
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| container_title | Nephrology, dialysis, transplantation |
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| creator | Ruggiero, Barbara Vivarelli, Marina Gianviti, Alessandra Pecoraro, Carmine Peruzzi, Licia Benetti, Elisa Ventura, Giovanna Pennesi, Marco Murer, Luisa Coppo, Rosanna Emma, Francesco |
| description | 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.
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 |
| doi_str_mv | 10.1093/ndt/gfw230 |
| format | article |
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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.
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.
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.</description><identifier>ISSN: 0931-0509</identifier><identifier>EISSN: 1460-2385</identifier><identifier>DOI: 10.1093/ndt/gfw230</identifier><identifier>PMID: 27270291</identifier><language>eng</language><publisher>England</publisher><subject>Adolescent ; Age of Onset ; Child ; Disease Progression ; Female ; Glomerular Filtration Rate ; Glomerulonephritis - diagnosis ; Glomerulonephritis - etiology ; Glomerulonephritis - mortality ; Humans ; Kidney Diseases - diagnosis ; Kidney Diseases - etiology ; Kidney Diseases - mortality ; Lupus Erythematosus, Systemic - complications ; Lupus Nephritis - diagnosis ; Lupus Nephritis - etiology ; Lupus Nephritis - mortality ; Male ; Proteinuria - pathology ; Recurrence ; Remission Induction ; Retrospective Studies ; Risk Factors ; Survival Rate</subject><ispartof>Nephrology, dialysis, transplantation, 2017-07, Vol.32 (7), p.1194-1204</ispartof><rights>The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c389t-68159512652475095b1e81634f3f9d710b47d2d16a4cd68c6eb093904b4b301c3</citedby><cites>FETCH-LOGICAL-c389t-68159512652475095b1e81634f3f9d710b47d2d16a4cd68c6eb093904b4b301c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/27270291$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ruggiero, Barbara</creatorcontrib><creatorcontrib>Vivarelli, Marina</creatorcontrib><creatorcontrib>Gianviti, Alessandra</creatorcontrib><creatorcontrib>Pecoraro, Carmine</creatorcontrib><creatorcontrib>Peruzzi, Licia</creatorcontrib><creatorcontrib>Benetti, Elisa</creatorcontrib><creatorcontrib>Ventura, Giovanna</creatorcontrib><creatorcontrib>Pennesi, Marco</creatorcontrib><creatorcontrib>Murer, Luisa</creatorcontrib><creatorcontrib>Coppo, Rosanna</creatorcontrib><creatorcontrib>Emma, Francesco</creatorcontrib><title>Outcome of childhood-onset full-house nephropathy</title><title>Nephrology, dialysis, transplantation</title><addtitle>Nephrol Dial Transplant</addtitle><description>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.
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.
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.
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.</description><subject>Adolescent</subject><subject>Age of Onset</subject><subject>Child</subject><subject>Disease Progression</subject><subject>Female</subject><subject>Glomerular Filtration Rate</subject><subject>Glomerulonephritis - diagnosis</subject><subject>Glomerulonephritis - etiology</subject><subject>Glomerulonephritis - mortality</subject><subject>Humans</subject><subject>Kidney Diseases - diagnosis</subject><subject>Kidney Diseases - etiology</subject><subject>Kidney Diseases - mortality</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Lupus Nephritis - diagnosis</subject><subject>Lupus Nephritis - etiology</subject><subject>Lupus Nephritis - mortality</subject><subject>Male</subject><subject>Proteinuria - pathology</subject><subject>Recurrence</subject><subject>Remission Induction</subject><subject>Retrospective Studies</subject><subject>Risk Factors</subject><subject>Survival Rate</subject><issn>0931-0509</issn><issn>1460-2385</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2017</creationdate><recordtype>article</recordtype><recordid>eNo9kEtLxDAURoMoTh3d-AOkSxHi3JukabOUwRcMzEbXpc3DjrRNbVpk_r2RGV3dxT18HA4h1wj3CIqvejOtPtw343BCEhQSKONFdkqS-EQKGagFuQjhEwAUy_NzsmA5y4EpTAhu50n7zqbepbrZtabx3lDfBzulbm5b2vg52LS3QzP6oZqa_SU5c1Ub7NXxLsn70-Pb-oVuts-v64cN1bxQE5UFZipDJjMm8qiQ1WgLlFw47pTJEWqRG2ZQVkIbWWhp66irQNSi5oCaL8ntYXcY_ddsw1R2u6Bt21a9jU4lFkxKJRiDiN4dUD36EEbrymHcddW4LxHK30RlTFQeEkX45rg71501_-hfE_4Dm-ZgtA</recordid><startdate>20170701</startdate><enddate>20170701</enddate><creator>Ruggiero, Barbara</creator><creator>Vivarelli, Marina</creator><creator>Gianviti, Alessandra</creator><creator>Pecoraro, Carmine</creator><creator>Peruzzi, Licia</creator><creator>Benetti, Elisa</creator><creator>Ventura, Giovanna</creator><creator>Pennesi, Marco</creator><creator>Murer, Luisa</creator><creator>Coppo, Rosanna</creator><creator>Emma, Francesco</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20170701</creationdate><title>Outcome of childhood-onset full-house nephropathy</title><author>Ruggiero, Barbara ; Vivarelli, Marina ; Gianviti, Alessandra ; Pecoraro, Carmine ; Peruzzi, Licia ; Benetti, Elisa ; Ventura, Giovanna ; Pennesi, Marco ; Murer, Luisa ; Coppo, Rosanna ; Emma, Francesco</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c389t-68159512652475095b1e81634f3f9d710b47d2d16a4cd68c6eb093904b4b301c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Adolescent</topic><topic>Age of Onset</topic><topic>Child</topic><topic>Disease Progression</topic><topic>Female</topic><topic>Glomerular Filtration Rate</topic><topic>Glomerulonephritis - diagnosis</topic><topic>Glomerulonephritis - etiology</topic><topic>Glomerulonephritis - mortality</topic><topic>Humans</topic><topic>Kidney Diseases - diagnosis</topic><topic>Kidney Diseases - etiology</topic><topic>Kidney Diseases - mortality</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Lupus Nephritis - diagnosis</topic><topic>Lupus Nephritis - etiology</topic><topic>Lupus Nephritis - mortality</topic><topic>Male</topic><topic>Proteinuria - pathology</topic><topic>Recurrence</topic><topic>Remission Induction</topic><topic>Retrospective Studies</topic><topic>Risk Factors</topic><topic>Survival Rate</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ruggiero, Barbara</creatorcontrib><creatorcontrib>Vivarelli, Marina</creatorcontrib><creatorcontrib>Gianviti, Alessandra</creatorcontrib><creatorcontrib>Pecoraro, Carmine</creatorcontrib><creatorcontrib>Peruzzi, Licia</creatorcontrib><creatorcontrib>Benetti, Elisa</creatorcontrib><creatorcontrib>Ventura, Giovanna</creatorcontrib><creatorcontrib>Pennesi, Marco</creatorcontrib><creatorcontrib>Murer, Luisa</creatorcontrib><creatorcontrib>Coppo, Rosanna</creatorcontrib><creatorcontrib>Emma, Francesco</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Nephrology, dialysis, transplantation</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ruggiero, Barbara</au><au>Vivarelli, Marina</au><au>Gianviti, Alessandra</au><au>Pecoraro, Carmine</au><au>Peruzzi, Licia</au><au>Benetti, Elisa</au><au>Ventura, Giovanna</au><au>Pennesi, Marco</au><au>Murer, Luisa</au><au>Coppo, Rosanna</au><au>Emma, Francesco</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Outcome of childhood-onset full-house nephropathy</atitle><jtitle>Nephrology, dialysis, transplantation</jtitle><addtitle>Nephrol Dial Transplant</addtitle><date>2017-07-01</date><risdate>2017</risdate><volume>32</volume><issue>7</issue><spage>1194</spage><epage>1204</epage><pages>1194-1204</pages><issn>0931-0509</issn><eissn>1460-2385</eissn><abstract>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.
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.
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.
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.</abstract><cop>England</cop><pmid>27270291</pmid><doi>10.1093/ndt/gfw230</doi><tpages>11</tpages><oa>free_for_read</oa></addata></record> |
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| ispartof | Nephrology, dialysis, transplantation, 2017-07, Vol.32 (7), p.1194-1204 |
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| source | Oxford University Press:Jisc Collections:OUP Read and Publish 2024-2025 (2024 collection) (Reading list) |
| subjects | Adolescent Age of Onset Child Disease Progression Female Glomerular Filtration Rate Glomerulonephritis - diagnosis Glomerulonephritis - etiology Glomerulonephritis - mortality Humans Kidney Diseases - diagnosis Kidney Diseases - etiology Kidney Diseases - mortality Lupus Erythematosus, Systemic - complications Lupus Nephritis - diagnosis Lupus Nephritis - etiology Lupus Nephritis - mortality Male Proteinuria - pathology Recurrence Remission Induction Retrospective Studies Risk Factors Survival Rate |
| title | Outcome of childhood-onset full-house nephropathy |
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