IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?

IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?

IgA Nephropathy (IgAN) is the most prevalent biopsy-proven primary glomerular disease worldwide. Historically, thrombotic microangiopathy (TMA) was associated with IgAN in cases of severe hypertension or advanced chronic kidney disease, but recent data suggest that complement activation plays a crucial role in the development of TMA in IgAN. We report a case of Crescentic IgAN with complement-mediated TMA (C-TMA) in a 27-year old male patient with a pathological missense mutation in heterozygosity in the CFH gene and a rare variant in the C3 gene, treated with steroids, cyclophosphamide and plasmapheresis without recovery of kidney function. We also discuss other treatment possibilities and kidney transplant options. Additionally, we will review the latest advances that are enhancing our understanding of the association between IgAN and TMA

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Main Authors: Diniz,Hugo, Bandeira,Maria, Teresa,Ana, Besteiro,Bruno, Coimbra,João, Gomes,Filipa, Sampaio,Susana
Format: Digital revista
Language:English
Published: Sociedade Portuguesa de Nefrologia 2019
Online Access:http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000400010
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spelling oai:scielo:S0872-016920190004000102020-02-18IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?Diniz,HugoBandeira,MariaTeresa,AnaBesteiro,BrunoCoimbra,JoãoGomes,FilipaSampaio,Susana Complement Genetics Hemolytic Uremic Syndrome IgA Nephropathy Thrombotic Microangiopathies IgA Nephropathy (IgAN) is the most prevalent biopsy-proven primary glomerular disease worldwide. Historically, thrombotic microangiopathy (TMA) was associated with IgAN in cases of severe hypertension or advanced chronic kidney disease, but recent data suggest that complement activation plays a crucial role in the development of TMA in IgAN. We report a case of Crescentic IgAN with complement-mediated TMA (C-TMA) in a 27-year old male patient with a pathological missense mutation in heterozygosity in the CFH gene and a rare variant in the C3 gene, treated with steroids, cyclophosphamide and plasmapheresis without recovery of kidney function. We also discuss other treatment possibilities and kidney transplant options. Additionally, we will review the latest advances that are enhancing our understanding of the association between IgAN and TMAinfo:eu-repo/semantics/openAccessSociedade Portuguesa de NefrologiaPortuguese Journal of Nephrology & Hypertension v.33 n.4 20192019-12-01info:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000400010en10.32932/pjnh.2020.01.050
institution SCIELO
collection OJS
country Portugal
countrycode PT
component Revista
access En linea
databasecode rev-scielo-pt
tag revista
region Europa del Sur
libraryname SciELO
language English
format Digital
author Diniz,Hugo
Bandeira,Maria
Teresa,Ana
Besteiro,Bruno
Coimbra,João
Gomes,Filipa
Sampaio,Susana
spellingShingle Diniz,Hugo
Bandeira,Maria
Teresa,Ana
Besteiro,Bruno
Coimbra,João
Gomes,Filipa
Sampaio,Susana
IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
author_facet Diniz,Hugo
Bandeira,Maria
Teresa,Ana
Besteiro,Bruno
Coimbra,João
Gomes,Filipa
Sampaio,Susana
author_sort Diniz,Hugo
title IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
title_short IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
title_full IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
title_fullStr IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
title_full_unstemmed IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?
title_sort iga nephropathy with thrombotic microangiopathy: is this secondary thrombotic microangiopathy or iga nephropathy-triggered atypical hemolytic uremic syndrome?
description IgA Nephropathy (IgAN) is the most prevalent biopsy-proven primary glomerular disease worldwide. Historically, thrombotic microangiopathy (TMA) was associated with IgAN in cases of severe hypertension or advanced chronic kidney disease, but recent data suggest that complement activation plays a crucial role in the development of TMA in IgAN. We report a case of Crescentic IgAN with complement-mediated TMA (C-TMA) in a 27-year old male patient with a pathological missense mutation in heterozygosity in the CFH gene and a rare variant in the C3 gene, treated with steroids, cyclophosphamide and plasmapheresis without recovery of kidney function. We also discuss other treatment possibilities and kidney transplant options. Additionally, we will review the latest advances that are enhancing our understanding of the association between IgAN and TMA
publisher Sociedade Portuguesa de Nefrologia
publishDate 2019
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-01692019000400010
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