Gantzel Rasmus Hvidbjerg, Bagge Carina Nørskov, Villadsen Gerda Elisabeth, Rex Karsten Fleischer, Grønbæk Henning, Pedersen Michael Lynge
Steno Diabetes Center Greenland, Queen Ingrid's Hospital, Nuuk, Greenland.
Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus, Denmark.
Int J Circumpolar Health. 2024 Dec;83(1):2327693. doi: 10.1080/22423982.2024.2327693. Epub 2024 Mar 11.
Autoimmune liver diseases are rare serious diseases causing chronic inflammation and fibrosis in the liver parenchyma and bile ducts. Yet, the prevalence and burden of autoimmune liver diseases are largely unexplored in Arctic native populations. We investigated the prevalence and management of autoimmune liver diseases in Greenland using nationwide cross-sectional register data and subsequent medical chart reviews validating diagnoses and extracting liver histology examinations and medical treatments. The overall prevalence of autoimmune liver diseases in Greenland was 24.6 per 100,000 (95% CI: 14.7-41.3). This was based on 7 patients with autoimmune hepatitis (AIH) (12.3 per 100,000), 3 patients with primary biliary cholangitis (PBC) (5.3 per 100,000), 4 patients with AIH/PBC overlap disease (7.0 per 100,000), and no patients with primary sclerosing cholangitis. All diagnoses were confirmed by liver histology examinations. Medical treatments adhered to internal recommendations and induced complete remission in most patients with AIH, and complete or partial remission in 1 patient with PBC and 3 patients with AIH/PBC overlap disease. One patient had established cirrhosis at the time of diagnosis, while 2 patients progressed to cirrhosis. In conclusion, the prevalence of autoimmune liver diseases was lower in Greenland than in Scandinavia and among Alaska Inuit.
自身免疫性肝病是罕见的严重疾病,可导致肝实质和胆管出现慢性炎症及纤维化。然而,北极原住民人群中自身免疫性肝病的患病率和疾病负担在很大程度上仍未得到研究。我们利用全国性横断面登记数据以及随后对病历的复查来验证诊断并提取肝脏组织学检查和药物治疗信息,从而对格陵兰岛自身免疫性肝病的患病率及治疗情况展开调查。格陵兰岛自身免疫性肝病的总体患病率为每10万人中有24.6例(95%置信区间:14.7 - 41.3)。这一数据基于7例自身免疫性肝炎(AIH)患者(每10万人中有12.3例)、3例原发性胆汁性胆管炎(PBC)患者(每10万人中有5.3例)、4例AIH/PBC重叠综合征患者(每10万人中有7.0例),且无原发性硬化性胆管炎患者。所有诊断均通过肝脏组织学检查得以证实。药物治疗遵循内部建议,多数AIH患者实现了完全缓解,1例PBC患者以及3例AIH/PBC重叠综合征患者实现了完全或部分缓解。1例患者在诊断时已发展为肝硬化,另有2例患者进展为肝硬化。总之,格陵兰岛自身免疫性肝病的患病率低于斯堪的纳维亚半岛以及阿拉斯加因纽特人群。