Year 2017 / Volume 109 / Number 6
Case Report
The clinical extremes of autoimmune cholangitis

465-468

DOI: 10.17235/reed.2017.4167/2015

Sara Campos, Dário Gomes, Maria Augusta Cipriano, Carlos Sofia,

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.
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Campos S, Gomes D, Cipriano M, Sofia C. The clinical extremes of autoimmune cholangitis. 4167/2015


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Publication history

Received: 18/12/2015

Accepted: 17/12/2016

Online First: 03/04/2017

Published: 01/06/2017

Article revision time: 364 days

Article Online First time: 472 days

Article editing time: 531 days


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