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Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study
Umeå University, Faculty of Medicine, Department of Public Health and Clinical Medicine, Medicine.
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2008 (English)In: Scandinavian Journal of Gastroenterology, ISSN 0036-5521, Vol. 43, no 10, 1232-1240 p.Article in journal (Refereed) Published
Abstract [en]

OBJECTIVE: Autoimmune hepatitis (AIH) is a chronic liver disease, which if untreated can lead to cirrhosis and hepatic failure. The aim of the study was to investigate the incidence, prevalence, diagnostic tradition and clinical initial presentation of AIH.

MATERIAL AND METHODS: Analyses were performed in 473 patients identified as having probable or definite AIH. RESULTS: The incidence of AIH was 0.85/100,000 (95% CI 0.69-1.01) inhabitants, which is somewhat lower than reported previously. The point prevalence amounted to 10.7/100,000 (95% CI 8.8-13.1), and 76% of the cases were females. The age-related incidence curve was bimodal but men were found to have only one incidence peak in the late teens, whereas women had a peak after menopause. AIH was presented as a spectrum of clinical settings from detected "en passant" to acute liver failure. Almost 30% of patients already had liver cirrhosis at diagnosis. Autoantibodies indicative of AIH type 1 were found in 79% of cases. Other concomitant autoimmune diseases were frequently found (49%).

CONCLUSIONS: The incidence and prevalence figures confirm that AIH is a fairly uncommon disease in the Swedish population. Symptoms at presentation were unspecific, but almost half of the patients were jaundiced, with around 30% having liver cirrhosis. The majority of Swedish AIH patients had AIH type 1.

Place, publisher, year, edition, pages
Informa Healthcare, 2008. Vol. 43, no 10, 1232-1240 p.
Keyword [en]
ANA, autoimmune hepatitis, cirrhosis, epidemiology, initial presentation, SMA
National Category
Gastroenterology and Hepatology
URN: urn:nbn:se:umu:diva-18535DOI: 10.1080/00365520802130183ISI: 000259318200011PubMedID: 18609163OAI: diva2:160364
Available from: 2009-02-13 Created: 2009-02-13 Last updated: 2015-09-07Bibliographically approved
In thesis
1. Autoimmune hepatitis in Sweden
Open this publication in new window or tab >>Autoimmune hepatitis in Sweden
2009 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Autoimmune hepatitis (AIH) was identified as an entity by the Swedish professor Jan Waldenström in the 1950s. It was then denoted lupoid hepatitis, characterized by liver inflammation and most often affecting young women. During the years the diagnosis has become more defined (as the non A non B hepatitis has been identified as Hepatitis C) and now can be safely separated from other diseases with liver inflammation. Studies of epidemiological data and long term prognosis have been scarce in the literature. Within a collaboration between the university hospitals in Sweden, we collected what we believe is the largest cohort in the world of patients with AIH. Data from the medical records of 473 individuals was, after AIH-score calculations where the diagnosis was confirmed, collected in a data base, in which most of the analysis was done. Data from the Swedish national registers of cancer, death cause, and birth register was searched for these patients as well as controls. The aim of the thesis was to explore epidemiological and clinical outcome of AIH.The onset of AIH may be at any age, but the incidence seems to increase after 50 years of age; 75% are females, the overall incidence (0.85/ 100,000 inhabitants and year) and prevalence (11/100,000 inhabitants) are figures that are within the range of another but smaller Scandinavian study. Approximately 30 % had cirrhosis already at diagnosis and 87% displayed at some time positive auto-antibodies indicating AIH (Smooth muscle ab and or antinuclear ab).  Indications of future risk for liver transplantation or death is an advanced AIH at diagnosis with liver cirrhosis, decompensated liver disease, elevated PK INR as well as age. Acute hepatitis-like onset seems to carry a lower risk for later liver transplantation or death. Current Swedish national therapy traditions with immune suppression seem to be well tolerated. Five and ten years overall life expectancy does not differ from controls. Thirty-five women gave birth to 63 children, for 3 after liver transplantation of the mother. Thirteen of the women had liver cirrhosis. Current pharmacological treatment seems to be safe both for the patient and the foetus. Thirty percent of the patients experienced flair after delivery. It has been supposed that there is an overrisk for hepatocellular cancer (HCC) associated with AIH. Our figures are the first in the world to be presented that confirms a twenty-three fold overrisk (95% Confidence Interval 7.5-54.3) for hepatobiliar cancer. We found as well an overrisk of non-Hodgkin lymphomas of 13.09 (95% CI 4.2-30.6).Conclusion:  Our epidemiological results confirm that AIH is a fairly uncommon disease, and that many already at time of diagnosis have an advanced disease with liver cirrhosis. There is a clear overrisk for HCC and lymphoma. For those women with AIH who become pregnant the prognosis for the child as well as for the mother is good, even for those women who already have compensated cirrhosis. There is a risk for relapse after delivery. The overall survival for AIH patients with current therapy is good.

Place, publisher, year, edition, pages
Umeå: Umeå universitet, 2009. 60 p.
Umeå University medical dissertations, ISSN 0346-6612 ; 1233
Autoimmune hepatitis, epidemiology, cirrhosis, prognosis, thiopurines, pregnancy, breast feeding, relapse, hepatocellular cancer, lymphoma
National Category
Gastroenterology and Hepatology
Research subject
urn:nbn:se:umu:diva-18545 (URN)978-91-7264-701-5 (ISBN)
Public defence
2009-03-20, Sal B, Byggnad 1D, Tandläkarhögskolan, Norrlands universitetssjukhus, Umeå, 09:00 (Swedish)
Available from: 2009-03-04 Created: 2009-02-13 Last updated: 2015-09-07Bibliographically approved

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