Usefulness of corticosteroids for the treatment of severe and fulminant forms of autoimmune hepatitis
Usefulness of corticosteroids for the treatment of severe and fulminant forms of autoimmune hepatitis
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DOI：
10.1002/lt.21036
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发表时间：
2007-07-01
影响因子：
4.6
通讯作者：
Samuel, Didier
中科院分区：
文献类型：
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作者：
Ichai, Philippe;Duclos-Vallee, Jean-Charles;Samuel, Didier
免疫抑制治疗，特别是皮质类固醇联合或不联合硫唑嘌呤，可以使80%以上的自身免疫性肝炎（AIH）患者获得缓解。相比之下，皮质类固醇治疗严重形式的AIH的有用性仍然是一个争论的主题。1986年至2005年，16例（14例女性，2例男性;平均年龄：36.6 ± 13.1岁）因1型AIH（n = 13）或2型AIH（n = 3）而出现急性、重度或暴发性疾病的患者入住我们的肝脏重症监护室。入院时，16例患者中有10例（62.5%）出现脑病。中位国际标准化比值（INR）、胆红素、丙氨酸氨基转移酶（ALT）和肌酐值分别为5.36（范围：1.7-12.2）、425 μ mol/L（范围：278-850）、678 IU/L（范围：60-2867）和72 μ mol/L（范围：52-133）。共有12例患者接受了皮质类固醇治疗：8例患者之前在转诊中心开始接受治疗，中位数为2.5天（范围：1-89天），4例患者在入院时开始接受该治疗（中位数：2天;范围：0-5天）。4名患者因AIH迅速恶化而未接受治疗。治疗前12例患者中有4例患有脑病。皮质类固醇治疗的中位持续时间为7天（范围：2-135天）。在16例患者中，13例接受了肝移植（LT）（81%），当时所有患者均患有脑病。移植时INR、总胆红素和ALT的中位值分别为7.2（范围：3.3-15.9）、400 μ mol/L（范围：301-550）和706 IU/L（范围：69- 1，932）。所有接受皮质类固醇治疗的患者在移植时均发生了临床（脑病）和生化（终末期肝病模型[MELD]评分）恶化。组织学结果未显示任何潜在慢性肝病的特征。在13例接受移植的患者中，10例既往接受过皮质类固醇治疗。在2例接受皮质类固醇治疗的非移植患者中，仅1例患者观察到临床改善。3例皮质类固醇治疗患者发生严重脓毒症并发症（革兰氏阴性败血症2例;播散性曲霉菌病1例）。9例接受治疗的患者仍然存活; 1例在肝移植（LT）后死亡（AIH复发，急性胰腺炎，败血症），1例在没有LT的情况下存活，1例在没有LT的情况下死亡。在未接受治疗的患者中，3例在LT后存活，1例在没有LT的情况下死亡。总之，皮质类固醇治疗在严重和暴发性AIH中的益处不大;它可能有利于败血症并发症，不应延迟LT。
Immunosuppressive therapy, and particularly corticosteroids with or without azathioprine, can achieve a remission in more than 80% of patients with autoimmune hepatitis (AIH). By contrast, the usefulness of corticosteroid therapy in severe forms of AIH remains a subject of debate. Between 1986 and 2005, 16 patients (14 females, 2 males; mean age: 36.6 +/- 13.1 yr) presenting with acute, severe, or fulminant disease due to type 1 AIH (n = 13) or type 2 AIH (n = 3) were admitted to our liver intensive care unit. At admission, 10 of 16 (62.5%) patients presented with encephalopathy. Median international normalized ratio (INR), bilirubin, alanine aminotransferase (ALT), and creatinine values were 5.36 (range, 1.7-12.2), 425 mu mol/L (range, 278-850), 678 IU/L (range, 60-2867), and 72 mu mol/L (range, 52-133), respectively. A total of 12 patients received corticosteroid therapy: 8 had started in the referring center a median of 2.5 days (range, 1-89) previously, and this therapy was initiated in 4 patients at their admission to our unit (median: 2 days; range: 0-5). Four patients were not treated because of a rapid deterioration in their AIH. Before treatment, 4 of 12 patients had been suffering from encephalopathy. The median duration of corticosteroid therapy was 7 days (range: 2-135). Of 16 patients, 13 underwent liver transplantation (LT) (81%), at which time all were encephalopathic. Median values for INR, total bilirubin, and ALT were 7.2 (range: 3.3-15.9), 400 mu mol/L (range: 301-550), and 706 IU/L (range: 69-1,932), respectively, at the time of transplantation. All patients treated with corticosteroids had experienced a clinical (encephalopathy) and biochemical (Model for End-Stage Liver Disease [MELD] score) deterioration at the time of transplantation. Histological findings did not reveal any features of underlying chronic liver disease. Of the 13 patients undergoing transplantation, 10 had received prior corticosteroid therapy. Of the 2 nontransplanted patients treated with corticosteroids, a clinical improvement was observed in only 1 patient. Severe septic complications occurred in 3 patients under corticosteroid therapy (gram-negative septicemia In = 2; disseminated aspergillus In = 1). Nine of the treated patients are still alive; 1 died after liver transplantation (LT) (recurrence of AIH, acute pancreatitis, sepsis), 1 survived without LT, and 1 died without LT. Among the untreated patients, 3 survived after LT and 1 died without LT. In conclusion, corticosteroid therapy is of little benefit in severe and fulminant forms of AIH; it may favor septic complications and should not delay LT.