Does the diagnosis of primary biliary cirrhosis or autoimmune cholangitis depend on the 'phase' of the disease?
BACKGROUND
It is unclear whether autoimmune cholangitis (AIC) is a separate disease entity or primary biliary cirrhosis (PBC) without antimitochondrial antibodies (AMA) since fluctuation of AMA titres by immunofluorescence (IF) is often observed during the course of PBC. The aim of this study was to determine the serial changes in AMA profiles during the course of initially diagnosed PBC or AIC.


METHODS
In this prospective study, 32 patients with PBC or AIC were followed-up for at least 20 months and tested for AMA by IF, enzyme-linked immunosorbent assay (ELISA), and immunoblotting (IB).


RESULTS
When positive AMA result was defined as 'AMA by IF positive', 'AMA by IF and/or ELISA positive', and 'AMA by IB positive', the diagnosis of PBC or AIC did not change in 78%, 91%, and 97%, respectively, throughout follow-up. However, the diagnosis changed in one patient, and three patients were diagnosed as AIC throughout follow-up, despite the use of all three assays.


CONCLUSIONS
Our results suggested that the diagnosis of PBC and AIC was dependent on the 'phase' of the respective disease in 22% of the patients when negative AMA result was defined as 'AMA by IF negative'. This may result in recommending IB analysis before making the diagnosis of AIC.
Kadokawa
Yoshiko
Y
Second Department of Internal Medicine, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan.
Omagari
Katsuhisa
K
Ohba
Kazuo
K
Masuda
Jun-Ichi
J
Hazama
Hiroaki
H
Kinoshita
Hideki
H
Ohnita
Ken
K
Mizuta
Yohei
Y
Tanioka
Hajime
H
Imanishi
Takeo
T
Kohno
Shigeru
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000974:Antibodies, Antinuclear / Q000032:analysis / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D015153:Blotting, Western; D002761:Cholangitis / Q000175:diagnosis* / Q000276:immunology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis* / Q000503:physiopathology; D008111:Liver Function Tests; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D011336:Probability; D011446:Prospective Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
D000974:Antibodies, Antinuclear