PBC, Primary biliary cirrhosis

PBC,原发性胆汁性肝硬化
  • 文章类型: Journal Article
    自身免疫性肝炎(AIH)的急性加重对诊断提出了重大挑战,因为它可以模拟急性病毒性肝炎,尤其是在没有自身抗体和高丙种球蛋白血症的情况下。
    为了确定临床,实验室,AIH急性加重患者的组织病理学特征和治疗反应。
    对8年(2008-2016年)诊断为AIH急性加重的16例患者的回顾性分析。
    在111名诊断为AIH的患者中,16例(14.4%)患者被诊断为AIH急性加重.所有患者均为女性,中位年龄为35岁。9名患者(56%)患有1型AIH,7名(44%)患者被诊断为血清阴性AIH。所有16例(100%)患者在就诊时都有急性病毒性肝炎样疾病。胆红素中位数为4.2mg/dl(范围,2.2-20),天冬氨酸转氨酶为568IU/L(范围,390-908),丙氨酸转氨酶为430IU/L(范围,257-1026)和血清碱性磷酸酶为395IU/L(范围,112-890)在症状期。组织病理学检查显示10例(71.4%)患者有潜在的慢性肝炎,只有2例(14.2)患者的纤维化和2例(14.2%)的肝硬化。所有16例(100%)患者均接受了类固醇和硫唑嘌呤的联合治疗。13例(81%)患者实现了生化完全缓解,3例(19%)患者实现了部分缓解,其中1例(6%)患者因肝硬化并发症而死于疾病。
    在没有阳性病毒标志物的情况下,模拟急性病毒性肝炎的无法解释的急性肝炎患者应考虑AIH的急性加重。在这种情况下,通过免疫血清学标志物和肝活检的评估可以诊断AIH急性加重。
    UNASSIGNED: Acute exacerbation of Autoimmune Hepatitis (AIH) poses a significant challenge for diagnosis as it can mimic acute viral hepatitis especially in absence of autoantibodies and hypergammaglobulinemia.
    UNASSIGNED: To determine the clinical, laboratory, histopathological characteristics and response to treatment in AIH patients with acute exacerbation.
    UNASSIGNED: A retrospective analysis of 16 patients with acute exacerbation of AIH diagnosed over a period of eight years (2008-2016).
    UNASSIGNED: Out of the 111 patients diagnosed with AIH, acute exacerbation of AIH was diagnosed in 16 (14.4%) patients. All patients were females with median age of 35 years. Nine patients (56%) had Type 1 AIH and seven (44%) patients were diagnosed with seronegative AIH. All 16 (100%) patients had acute viral hepatitis like illness at presentation. The median bilirubin was 4.2 mg/dl (range, 2.2-20), aspartate transaminase was 568 IU/L (range, 390-908), alanine transaminase was 430 IU/L (range, 257-1026) and serum alkaline phosphatase was 395 IU/L (range, 112-890) during symptomatic period. The histopathological examination showed underlying chronic hepatitis in 10 (71.4%) patients, only fibrosis in 2 (14.2) patients and cirrhosis with activity in 2 (14.2%). All 16 (100%) patients were treated with a combination of steroids and azathioprine. Thirteen (81%) patients achieved complete biochemical remission and three (19%) patients achieved partial remission out of which one (6%) patient succumbed to illness because of the complications of cirrhosis.
    UNASSIGNED: A suspicion of acute exacerbation of AIH should be considered in patients with unexplained acute hepatitis mimicking acute viral hepatitis in the absence of positive viral markers. Through evaluation with immunoserological markers and liver biopsy can clinch the diagnosis of acute exacerbation of AIH in such cases.
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