The clinical extremes of autoimmune cholangitis
Rev. esp. enferm. dig
; 109(6): 465-468, jun. 2017. ilus
Article
in English
| IBECS
| ID: ibc-163265
Responsible library:
ES1.1
Localization: BNCS
ABSTRACT
Autoimmune cholangitis (AIC) was first described in 1987 as immunocholangitis in three women who presented with signs and symptoms of primary biliary cholangitis (PBC), but who were antimitochondrial (AMA) negative and antinuclear antibodies (ANA) positive, and responded to immunosuppressive therapy with azathioprine and prednisolone (1). AIC is a rare chronic cholestatic inflammatory disease characterized by the presence of high ANA or smooth muscle antibodies (SMA) but AMA seronegativity. Histologically, AIC exhibits bile duct injury (2). In terms of therapeutics, in addition to response to ursodeoxycholic acid, a prompt response to corticosteroids has also been reported in earlier stages, distinguishing it from PBC. Herein the authors describe two cases with mixed signs of PBC and autoimmune hepatitis (AIH). The diagnostic differentiation between these diseases (AIC, PBC and AIH) is essential because of the different therapeutic strategies. Our cases highlight the importance of clinician awareness of the autoimmune spectrum of liver diseases (AU)
RESUMEN
No disponible
Full text:
Available
Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Biopsy
/
Autoimmunity
/
Cholangitis
/
Hepatitis, Autoimmune
Type of study:
Diagnostic study
Limits:
Adult
/
Female
/
Humans
Language:
English
Journal:
Rev. esp. enferm. dig
Year:
2017
Document type:
Article
Institution/Affiliation country:
Centro Hospitalar e Universitário de Coimbra/Portugal