IgG4-related disease and idiopathic membranous nephropathy: "The clothes do not make the man"â©.
Clin Nephrol
; 86 (2016)(12): 345-348, 2016 Dec.
Article
en En
| MEDLINE
| ID: mdl-27737530
ABSTRACT
This case report describes a patient with a previous history of autoimmune pancreatitis secondary to IgG4-related disease, who developed an overt nephrotic syndrome due to membranous nephropathy, surprisingly idiopathic. In all the previously described cases with both concurrent diseases, membranous nephropathy was considered to be secondary to the IgG4-related disease based on the absence of anti-PLA2R1 autoantibodies, and nephrotic syndrome usually remitted after treatment with steroids alone. However, in our patient positivity of serum anti-PLA2R1 autoantibodies together with a normal serum IgG4 level, and the absence of the other most commonly associated diseases were compatible with an idiopathic membranous nephropathy. Combination treatment with steroids and cyclophosphamide was successful. We hypothesize about causality or coincidental diseases and the importance of a correct classification.â©.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Pancreatitis
/
Enfermedades Autoinmunes
/
Glomerulonefritis Membranosa
/
Receptores de Fosfolipasa A2
/
Síndrome Nefrótico
Límite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
Clin Nephrol
Año:
2016
Tipo del documento:
Article