A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course / 대한내과학회지
Korean Journal of Medicine
;
: 182-186, 2014.
Artigo
em Coreano
| WPRIM
| ID: wpr-226798
ABSTRACT
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare disease characterized by increased serum levels of IgG4 and dense infiltration of IgG4-positive plasma cells with fibrosis in the bile duct wall. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP) and typically shows an excellent response to steroid therapy. Despite recent progress in understanding the clinical presentation of IgG4-SC, its diagnosis still remains a challenge, particularly if it manifests as an isolated hilar stricture with normal serum IgG4 concentrations. In this article, we report the case of a 75-year-old man with IgG4-SC in whom it was difficult to distinguish hilar cholangiocarcinoma due to the normal serum IgG4 concentration and no accompanying AIP. He had an indolent clinical course and showed slow progression of a bile duct lesion over 6 years.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Pancreatite
/
Plasmócitos
/
Ductos Biliares
/
Fibrose
/
Imunoglobulina G
/
Imunoglobulinas
/
Colangite Esclerosante
/
Colangiocarcinoma
/
Constrição Patológica
/
Doenças Raras
Tipo de estudo:
Estudo diagnóstico
Limite:
Idoso
/
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Medicine
Ano de publicação:
2014
Tipo de documento:
Artigo
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