Autoimmune cholangitis within the spectrum of autoimmune liver disease

Autoimmune hepatitis with incidental histologic features of bile duct injury

“…Reclassification of these patients as a variant syndrome based solely on the histologic findings in a single biopsy specimen may be premature, especially if the designation suggests a different natural history or dictates an alternative therapy. [12][13][14][15] Our findings underscore the need for codified diagnostic criteria for the variant syndromes and a complete description of natural history and treatment response. Biliary changes may be focal, transient, and/or inconsequential in nature in the context of a predominantly immune-mediated hepatocellular injury.…”

“…Our patients with biliary changes may constitute a variant form. [12][13][14][15] They differ, however, from other descriptions in that they satisfy international criteria for autoimmune hepatitis, and they respond well to corticosteroids, achieving clinical, laboratory, and histologic remission as commonly as patients without biliary changes. Reclassification of these patients as a variant syndrome based solely on the histologic findings in a single biopsy specimen may be premature, especially if the designation suggests a different natural history or dictates an alternative therapy.…”

“…These patients had been part of an earlier study comparing the histologic features of autoimmune cholangitis to classical autoimmune hepatitis. 13 Seventy patients (83%) were women, and their mean age at presentation was 45 Ϯ 2 years (range, 13-75 years). Eighteen patients (21%) had antinuclear antibodies (ANA); 19 patients (23%) had smooth muscle antibodies (SMA); and 47 patients (56%) had both ANA and SMA at presentation.…”

“…12 In contrast, destructive cholangitis and/or ductopenia are findings incompatible with the diagnosis of autoimmune hepatitis. Their occurrence has generated alternative diagnoses, such as autoimmune cholangitis 13 or an autoimmune variant syndrome characterized by overlapping features of autoimmune hepatitis and primary biliary cirrhosis or primary sclerosing cholangitis. [14][15][16] Christofferson et al first described bile duct changes in chronic hepatitis, but these studies were performed before the era of concise diagnostic classifications and confident screening methods for viral infection.…”

Cholestatic liver diseases: primary biliary cirrhosis

“…Nach 10 Jahren UDC-Therapie entwickelten Patienten im Stadium I zu 17%, im Stadium II zu 27% und im Stadium III zu 76% eine Leberzirrhose (6). Prädiktive Faktoren für die Entwicklung einer Leberzirrhose waren erhöhte Bilirubinwerte, erniedrigtes Serum Albumin und ausgeprägtere "Mottenfraß-Nekrosen" in der histologischen Untersuchung (6) Therapien scheinen bei der AIC eine höhere Ansprechrate aufzuweisen als bei der PBC (7,23).…”