Complement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report.
Atypical hemolytic uremic syndrome
Complement-mediated thrombotic microangiopathy
Glomerular disease
Pregnancy
Systemic lupus erythematosus
Thrombotic microangiopathy
Journal
Case reports in nephrology and dialysis
ISSN: 2296-9705
Titre abrégé: Case Rep Nephrol Dial
Pays: Switzerland
ID NLM: 101636294
Informations de publication
Date de publication:
Historique:
received:
06
05
2020
accepted:
20
09
2020
entrez:
12
5
2021
pubmed:
13
5
2021
medline:
13
5
2021
Statut:
epublish
Résumé
Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. We present an important case of a pregnant 23-year-old Hispanic female who presented in mid-gestation (21 weeks) with an initial diagnosis of systemic lupus erythematosus (SLE) complicated by aHUS. She met clinical criteria for aHUS on presentation and was found to have a pathogenic CFHR1-3 homozygous deletion. She has been treated with intravenous and oral steroids, cyclophosphamide, subsequently also with plasma exchange, and finally with eculizumab with partial improvement in renal function. This case adds to the emerging literature showing that SLE and aHUS (or complement-mediated TMA) can be successfully treated with C5 blockade.
Identifiants
pubmed: 33977097
doi: 10.1159/000512227
pii: cnd-0011-0095
pmc: PMC8077465
doi:
Types de publication
Case Reports
Langues
eng
Pagination
95-102Informations de copyright
Copyright © 2021 by S. Karger AG, Basel.
Déclaration de conflit d'intérêts
R.M.H. and R.B. are paid speakers and consultants for Alexion Pharmaceuticals.
Références
Thromb J. 2019 Sep 9;17:18
pubmed: 31516395
Pregnancy Hypertens. 2018 Apr;12:29-34
pubmed: 29674195
Blood. 2009 Sep 17;114(12):2439-47
pubmed: 19528535
J Am Soc Nephrol. 2011 Feb;22(2):285-95
pubmed: 21148254
Curr Opin Nephrol Hypertens. 2019 May;28(3):278-287
pubmed: 30865166
PLoS Genet. 2007 Mar 16;3(3):e41
pubmed: 17367211
Ther Adv Hematol. 2015 Aug;6(4):171-85
pubmed: 26288712
Obstet Gynecol. 2020 Jan;135(1):46-58
pubmed: 31809447
Blood Adv. 2018 Aug 28;2(16):2090-2094
pubmed: 30131343
Kidney Int. 2006 Aug;70(3):423-31
pubmed: 16775594
J Int Med Res. 2019 Aug;47(8):4027-4032
pubmed: 31364428
J Blood Med. 2016 Apr 12;7:39-72
pubmed: 27110144
N Engl J Med. 2013 Jun 6;368(23):2169-81
pubmed: 23738544