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未经治疗的1型自身免疫性肝炎的特征及后果。

Features and consequences of untreated type 1 autoimmune hepatitis.

作者信息

Czaja Albert J

机构信息

Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.

出版信息

Liver Int. 2009 Jul;29(6):816-23. doi: 10.1111/j.1478-3231.2008.01904.x. Epub 2008 Oct 14.

Abstract

BACKGROUND/AIM: The existence of a mild form of autoimmune hepatitis that does not require treatment remains controversial. The aim of this study was to determine the existence, characteristics and outcome of this form.

METHODS

Untreated patients with type 1 autoimmune hepatitis who did not satisfy pre-established criteria for severe disease were identified retrospectively and compared with treated patients.

RESULTS

Twenty-one of 282 patients (7%) who were evaluated during a 32-year period did not receive corticosteroid treatment. These asymptomatic patients constituted 15% of 137 patients who satisfied similar criteria for mild disease. Untreated patients with mild disease were indistinguishable from 116 treated patients with mild disease, and they differed from 145 treated patients with severe disease only by the pre-established features that defined disease severity. The eight untreated patients with follow-up assessments satisfied remission criteria less commonly than treated patients with mild or severe disease during 77+/-31 months of observation (12 vs 63%, P=0.006), and they had a lower 10-year survival (67 vs 98%, P=0.01). Four patients did improve spontaneously albeit short of remission criteria and remained well for 28+/-15 months (range, 5-73 months). Four patients worsened during 125+/-51 months of observation (range, 32-239 months), including two of three patients with cirrhosis who died of liver failure.

CONCLUSIONS

Mild type 1 autoimmune hepatitis does exist, and it may be as frequent as severe disease. Untreated patients with mild disease can improve spontaneously, but there are no confident indices by which to identify this subgroup and justify withholding treatment.

摘要

背景/目的:一种无需治疗的轻度自身免疫性肝炎的存在仍存在争议。本研究的目的是确定这种形式的存在、特征和结局。

方法

回顾性识别未接受治疗且不符合预先设定的严重疾病标准的1型自身免疫性肝炎患者,并与接受治疗的患者进行比较。

结果

在32年期间接受评估的282例患者中有21例(7%)未接受皮质类固醇治疗。这些无症状患者占137例符合轻度疾病类似标准患者的15%。未治疗的轻度疾病患者与116例接受治疗的轻度疾病患者无差异,且他们与145例接受治疗的严重疾病患者的区别仅在于预先确定的定义疾病严重程度的特征。8例接受随访评估的未治疗患者在77±31个月的观察期内达到缓解标准的比例低于接受治疗的轻度或重度疾病患者(12%对63%,P=0.006),且他们的10年生存率较低(67%对98%,P=0.01)。4例患者虽未达到缓解标准但确实自发改善,并在28±15个月(范围5 - 73个月)内保持良好状态。4例患者在125±51个月的观察期内(范围32 - 239个月)病情恶化,包括3例肝硬化患者中的2例死于肝衰竭。

结论

轻度1型自身免疫性肝炎确实存在,其发病率可能与严重疾病相同。未治疗的轻度疾病患者可自发改善,但没有可靠的指标来识别该亚组并证明可以不进行治疗。

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