Transitions between variant forms of primary biliary cirrhosis during long-term follow-up.
BACKGROUND
Conditions exhibiting features of two different autoimmune liver diseases are designated overlap syndromes. Variant forms display some, but not all, characteristics of a distinct autoimmune liver disease. We describe transitions over time between variant forms of PBC, i.e. AMA-negative PBC, autoimmune hepatitis (AIH)-PBC overlap and autoimmune cholangitis (AIC) in a large cohort of PBC patients in Sweden.


METHODS
We retrieved all patients with variant forms of PBC in six university hospitals in Sweden, covering 60% of the Swedish population. The diagnosis of PBC and its variants was based on laboratory findings and compatible histological features. The revised autoimmune hepatitis scoring system proposed by the International Autoimmune Hepatitis Group was used to establish the diagnosis of AIH.


RESULTS
In a population of 800 patients with PBC, we identified 35 (5%) variant forms; 25 patients with AIH-PBC overlap, 8 with AIC and 2 with AMA-negative PBC at the time of our study. The initial diagnoses were PBC (3 patients), AIH (3), AIH-PBC overlap (16), AIC (8) and AMA-negative PBC with (1) or without (4) concomitant AIH. The median follow-up was 125 (41-360) months. Immunosuppression and ursodeoxycholic acid induced a complete or good regression of increased aminotransferases in about half of the patients who were given one or both of these treatments.


CONCLUSIONS
Variant forms of PBC are seen in approximately 5% of PBC patients in Sweden. Transition between different forms may occur, emphasizing the value of repeat biopsies, but established overlapping AIH-PBC seems to be stable over time.
Lindgren
Stefan
S
Department of Medicine, University Hospital MAS, Malmö, Sweden. Stefan.Lindgren@med.lu.se
Glaumann
Hans
H
Almer
Sven
S
Bergquist
Annika
A
Björnsson
Einar
E
Broomé
Ulrika
U
Danielsson
Ake
A
Lebrun
Barbro
B
Prytz
Hanne
H
Olsson
Rolf
R
D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D001706:Biopsy; D002761:Cholangitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
D007166:Immunosuppressive Agents