关键词: acute on chronic liver disease aih -autoimmune hepatitis autoimmune flare up chronic liver disease (cld) live cirrhosis seronegative autoimmune hepatitis

来  源:   DOI:10.7759/cureus.55477   PDF(Pubmed)

Abstract:
BACKGROUND: Autoimmune hepatitis (AIH) is uncommon and predominantly affects females. Data on AIH from India are scanty. We retrospectively analyzed the spectrum and outcome of adults with AIH and compared it between male and female patients.
METHODS: AIH was diagnosed using a simplified AIH score. For suspected seronegative AIH, the revised score was used. Standard therapies for AIH and portal hypertension were administered and response was assessed at six months. Relapse rates and five-year mortality were also evaluated.
RESULTS: Of the 157 patients with AIH, 85 (male: female 25: 60) were included in the study. The median age at diagnosis was 46 (interquartile range (IQR) 32-55.5) years in males vs 45 (IQR 34.2-54) years in females (p=0.91). A similar proportion of male and female patients presented with cirrhosis, acute severe AIH, or AIH-related acute on chronic liver failure (ACLF); Extra-hepatic autoimmune diseases were less common in male patients (16% vs 35.5% p=0.02). Other laboratory and histological features were comparable in both groups. During the median follow-up period of 51 months (IQR 45-67 months). The biochemical and clinical response at six months were seen in 64% of male patients and 63.3% of female patients (p= 0.57). Of patients, 75% relapsed in the male AIH group (12 of 16 patients) after initial remission compared to 42% in the female group (p=0.02). Five-year mortality was 14.1%, and no patient developed hepatocellular carcinoma.
CONCLUSIONS: Male and female patients with AIH have similar clinical, biochemical, and histological profiles. More male patients relapsed after an initial response to therapy.
摘要:
背景:自身免疫性肝炎(AIH)并不常见,主要影响女性。来自印度的AIH数据很少。我们回顾性分析了成人AIH的频谱和结果,并将其在男性和女性患者之间进行了比较。
方法:使用简化的AIH评分诊断AIH。对于疑似血清阴性AIH,使用修订后的分数。给予AIH和门静脉高压症的标准疗法,并在六个月时评估反应。还评估了复发率和五年死亡率。
结果:在157例AIH患者中,研究中包括85(男性:女性25:60)。男性诊断的中位年龄为46岁(四分位距(IQR)32-55.5),女性为45岁(IQR34.2-54)(p=0.91)。男性和女性患者出现肝硬化的比例相似,急性重症AIH,或AIH相关的急性/慢性肝衰竭(ACLF);肝外自身免疫性疾病在男性患者中较少见(16%vs35.5%p=0.02)。两组的其他实验室和组织学特征具有可比性。中位随访期51个月(IQR45-67个月)。6个月时的生化和临床反应在64%的男性患者和63.3%的女性患者中观察到(p=0.57)。在患者中,男性AIH组(16例患者中有12例)在初始缓解后复发75%,而女性组为42%(p=0.02)。五年死亡率为14.1%,没有患者发生肝细胞癌。
结论:男性和女性AIH患者的临床表现相似,生物化学,和组织学特征。更多的男性患者在对治疗的初始反应后复发。
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