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Transitions between variant forms of primary biliary cirrhosis during long-term follow-up.

Lindgren, Stefan LU ; Glaumann, Hans; Almer, Sven; Bergquist, Annika; Björnsson, Einar; Broomé, Ulrika; Danielsson, Ake; Lebrun, Barbro; Prytz, Hanne LU and Olsson, Rolf LU (2009) In European Journal of Internal Medicine 20(4). p.398-402
Abstract
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... (More)
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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
European Journal of Internal Medicine
volume
20
issue
4
pages
398 - 402
publisher
Elsevier
external identifiers
  • wos:000268697800015
  • pmid:19524182
  • scopus:67349139257
ISSN
1879-0828
DOI
10.1016/j.ejim.2008.12.011
language
English
LU publication?
yes
id
b5205bf2-d874-4a2f-bd6d-667bcbe8b81b (old id 1434262)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/19524182?dopt=Abstract
date added to LUP
2009-07-03 15:15:26
date last changed
2017-08-06 04:44:19
@article{b5205bf2-d874-4a2f-bd6d-667bcbe8b81b,
  abstract     = {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.},
  author       = {Lindgren, Stefan and Glaumann, Hans and Almer, Sven and Bergquist, Annika and Björnsson, Einar and Broomé, Ulrika and Danielsson, Ake and Lebrun, Barbro and Prytz, Hanne and Olsson, Rolf},
  issn         = {1879-0828},
  language     = {eng},
  number       = {4},
  pages        = {398--402},
  publisher    = {Elsevier},
  series       = {European Journal of Internal Medicine},
  title        = {Transitions between variant forms of primary biliary cirrhosis during long-term follow-up.},
  url          = {http://dx.doi.org/10.1016/j.ejim.2008.12.011},
  volume       = {20},
  year         = {2009},
}