C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma

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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Main Authors: Costa,Joana Silva, Romãozinho,Catarina, Rodrigues,Luís, Marinho,Carol, Sousa,Vitor, Pratas,Jorge, Cipriano,Maria Augusta, Alves,Rui
Format: Digital revista
Language:English
Published: Sociedade Portuguesa de Nefrologia 2018
Online Access:http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011
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spelling oai:scielo:S0872-016920180001000112018-04-23C3 glomerulopathy: a rare kidney histological presentation of multiple myelomaCosta,Joana SilvaRomãozinho,CatarinaRodrigues,LuísMarinho,CarolSousa,VitorPratas,JorgeCipriano,Maria AugustaAlves,Rui complement C3 glomerulopathy multiple myeloma 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 hematologistsinfo:eu-repo/semantics/openAccessSociedade Portuguesa de NefrologiaPortuguese Journal of Nephrology & Hypertension v.32 n.1 20182018-03-01info:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011en
institution SCIELO
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country Portugal
countrycode PT
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access En linea
databasecode rev-scielo-pt
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region Europa del Sur
libraryname SciELO
language English
format Digital
author Costa,Joana Silva
Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
spellingShingle Costa,Joana Silva
Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
C3 glomerulopathy: a rare kidney histological presentation of multiple myeloma
author_facet Costa,Joana Silva
Romãozinho,Catarina
Rodrigues,Luís
Marinho,Carol
Sousa,Vitor
Pratas,Jorge
Cipriano,Maria Augusta
Alves,Rui
author_sort Costa,Joana Silva
title C3 glomerulopathy: a rare kidney histological presentation of 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
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
publisher Sociedade Portuguesa de Nefrologia
publishDate 2018
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692018000100011
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