Prevalence and longterm course of primary biliary cirrhosis in primary Sjögren's syndrome.
OBJECTIVE
To study the prevalence of primary biliary cirrhosis (PBC) and its progression in patients with primary Sjögren's syndrome (SS).


METHODS
We investigated 410 patients with primary SS, without history of liver disease, for the presence of PBC based on a retrospective review of clinical, biochemical, immunologic, and histologic data.


RESULTS
Thirty-six (8.8%) patients had cholestatic liver biochemistry. Of them, 21 (5.1%) had positive antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence, while 15 were AMA-negative. Ten of the 21 AMA-positive patients and 7 of the 15 AMA-negative patients were further investigated with liver biopsy, the result of which was compatible with PBC in all but one (AMA-negative) patient. Overall, 27 (6.6%) patients had definite (n=10), probable (n=11), or AMA-negative (n=6) PBC. Pathologically, most PBC lesions were stage 1. Five patients had a second liver biopsy, with no significant histological deterioration.


CONCLUSIONS
PBC is a rather uncommon development in patients with primary SS. The disease appears to be pathologically mild, with a propensity for slow progression, as assessed clinically, biochemically, and histologically.
Hatzis
Gregorios S
GS
Department of Pathophysiology, National University of Athens Medical School, and Department of Pathology, Red Cross Hospital, Athens, Greece.
Fragoulis
Georgios E
GE
Karatzaferis
Aggelos
A
Delladetsima
Ioanna
I
Barbatis
Calipso
C
Moutsopoulos
Haralampos M
HM
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology; D015995:Prevalence; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000453:epidemiology
D001323:Autoantibodies