C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma

Detalhes bibliográficos
Autor(a) principal: Costa,Joana Silva
Data de Publicação: 2018
Outros Autores: Romãozinho,Catarina, Rodrigues,Luís, Marinho,Carol, Sousa,Vitor, Pratas,Jorge, Cipriano,Maria Augusta, Alves,Rui
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011
Resumo: C3 Glomerulopathy is a rare disease caused by abnormal control of the alternative complement pathway, resulting in a predominant glomerular C3 deposition. Its association with multiple myeloma has been reported in recent literature. We present a case of a 66-year-old women, referred for a nephrology consultation with a stage 4 chronic kidney disease, microhematuria, leukocyturia, sub-nephrotic range proteinuria (1.3 g/24hours), albumin 3.7 g/dl and dyslipidemia. She had been previously studied in a Hematology consultation for a normocytic anemia and an IgG Kappa monoclonal gammopathy, with 16% of plasma cells in bone marrow aspiration, but no hypercalcemia or lytic bone lesions. Autoimmune tests (ANCA, ANA, Anti-dsDNA antibody), C3 and C4 were negative. Eight months later, the patient complained of hypertension and edema, and presented a mild decrease in serum C3 (0.86 g/L [0.90-1.80]) and progressive nephrotic proteinuria (from 4.5 to 6.1 g/24h), with hypoalbuminemia. A kidney biopsy was performed, and we found mild chronic non-specific glomerular and tubule-interstitial findings, on light microscopy, and mesangial C3 deposits (+++), without immunoglobulin deposits, on immunofluorescence. Immunofluorescence with protease-digested paraffin sections was also negative. Genetic study found no complement gene mutation. She was treated with prednisone 1mg/Kg/day, with a favorable clinical and laboratorial response, but whether this treatment is enough it is still being pondered between nephrologists and hematologists
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spelling C3 glomerulopathy: a rare kidney histological presentation of multiple myelomacomplementC3 glomerulopathymultiple myelomaC3 Glomerulopathy is a rare disease caused by abnormal control of the alternative complement pathway, resulting in a predominant glomerular C3 deposition. Its association with multiple myeloma has been reported in recent literature. We present a case of a 66-year-old women, referred for a nephrology consultation with a stage 4 chronic kidney disease, microhematuria, leukocyturia, sub-nephrotic range proteinuria (1.3 g/24hours), albumin 3.7 g/dl and dyslipidemia. She had been previously studied in a Hematology consultation for a normocytic anemia and an IgG Kappa monoclonal gammopathy, with 16% of plasma cells in bone marrow aspiration, but no hypercalcemia or lytic bone lesions. Autoimmune tests (ANCA, ANA, Anti-dsDNA antibody), C3 and C4 were negative. Eight months later, the patient complained of hypertension and edema, and presented a mild decrease in serum C3 (0.86 g/L [0.90-1.80]) and progressive nephrotic proteinuria (from 4.5 to 6.1 g/24h), with hypoalbuminemia. A kidney biopsy was performed, and we found mild chronic non-specific glomerular and tubule-interstitial findings, on light microscopy, and mesangial C3 deposits (+++), without immunoglobulin deposits, on immunofluorescence. Immunofluorescence with protease-digested paraffin sections was also negative. Genetic study found no complement gene mutation. She was treated with prednisone 1mg/Kg/day, with a favorable clinical and laboratorial response, but whether this treatment is enough it is still being pondered between nephrologists and hematologistsSociedade Portuguesa de Nefrologia2018-03-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011Portuguese Journal of Nephrology & Hypertension v.32 n.1 2018reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011Costa,Joana SilvaRomãozinho,CatarinaRodrigues,LuísMarinho,CarolSousa,VitorPratas,JorgeCipriano,Maria AugustaAlves,Ruiinfo:eu-repo/semantics/openAccess2024-02-06T17:04:57Zoai:scielo:S0872-01692018000100011Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:00.061816Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
title C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
spellingShingle C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
Costa,Joana Silva
complement
C3 glomerulopathy
multiple myeloma
title_short C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
title_full C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
title_fullStr C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
title_full_unstemmed C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
title_sort C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
author Costa,Joana Silva
author_facet Costa,Joana Silva
Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
author_role author
author2 Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Costa,Joana Silva
Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
dc.subject.por.fl_str_mv complement
C3 glomerulopathy
multiple myeloma
topic complement
C3 glomerulopathy
multiple myeloma
description C3 Glomerulopathy is a rare disease caused by abnormal control of the alternative complement pathway, resulting in a predominant glomerular C3 deposition. Its association with multiple myeloma has been reported in recent literature. We present a case of a 66-year-old women, referred for a nephrology consultation with a stage 4 chronic kidney disease, microhematuria, leukocyturia, sub-nephrotic range proteinuria (1.3 g/24hours), albumin 3.7 g/dl and dyslipidemia. She had been previously studied in a Hematology consultation for a normocytic anemia and an IgG Kappa monoclonal gammopathy, with 16% of plasma cells in bone marrow aspiration, but no hypercalcemia or lytic bone lesions. Autoimmune tests (ANCA, ANA, Anti-dsDNA antibody), C3 and C4 were negative. Eight months later, the patient complained of hypertension and edema, and presented a mild decrease in serum C3 (0.86 g/L [0.90-1.80]) and progressive nephrotic proteinuria (from 4.5 to 6.1 g/24h), with hypoalbuminemia. A kidney biopsy was performed, and we found mild chronic non-specific glomerular and tubule-interstitial findings, on light microscopy, and mesangial C3 deposits (+++), without immunoglobulin deposits, on immunofluorescence. Immunofluorescence with protease-digested paraffin sections was also negative. Genetic study found no complement gene mutation. She was treated with prednisone 1mg/Kg/day, with a favorable clinical and laboratorial response, but whether this treatment is enough it is still being pondered between nephrologists and hematologists
publishDate 2018
dc.date.none.fl_str_mv 2018-03-01
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dc.publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
publisher.none.fl_str_mv Sociedade Portuguesa de Nefrologia
dc.source.none.fl_str_mv Portuguese Journal of Nephrology & Hypertension v.32 n.1 2018
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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