Azathioprine-induced lung toxicity and efficacy of cyclosporin A in a young girl with type 2 autoimmune hepatitis.
Azathioprine-induced lung toxicity and efficacy of cyclosporin A in a young girl with type 2 autoimmune hepatitis.
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硫唑嘌呤引起的肺毒性和环孢菌素 A 对患有 2 型自身免疫性肝炎的年轻女孩的疗效。
DOI：
10.1097/00005176-200008000-00020
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发表时间：
2000
期刊：
影响因子：
--
通讯作者：
Philippe Labrune
中科院分区：
文献类型：
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作者：
Francis Perreaux;Delphine Zenaty;Frédérique Capron;P. Trioche;Michel Odièvre;Philippe Labrune
自身免疫性肝炎在儿童中很少见。根据血清中不同自身抗体的存在，可确定两种类型：抗核和/或平滑肌抗体（ANA/SMA）为1型，而肝肾微粒体（LKM-1）和/或抗肝胞质抗体定义为2型（1）。自身免疫性肝炎的治疗依赖于免疫抑制药物，通常是泼尼松和硫唑嘌呤，这两种药物经常联合使用，硫唑嘌呤起着减少类固醇的作用（2，3）。然而，这些药物可能有副作用，可能会限制甚至禁忌其管理。在可能的硫唑嘌呤相关副作用中，成人很少报告间质性肺炎（4-13），儿童更罕见（14）。诊断必须迅速确定，以便在不可逆的后遗症发生之前中断治疗。在这种情况下，即使皮质类固醇是有帮助的，他们可能无法控制疾病的进展本身，使其他药物的使用是必要的，其中环孢菌素A似乎是最有前途的（15）。我们报告一个年轻的女孩与自身免疫性慢性活动性肝炎，其中硫唑嘌呤治疗中断后，发展间质性肺炎。她成功地用环孢素A治疗了5年。几个月后复发，重新使用环孢菌素A后得到控制。
Autoimmune hepatitis is rare in children. Two types are identified according to the presence of different autoantibodies in the serum: the antinuclear and/or smooth muscle antibody (ANA/SMA) characterizes type 1, whereas the liver-kidney microsomal (LKM-1) and/or anti-liver cytosol antibody defines type 2 (1). The treatment of autoimmune hepatitis relies on immunosuppressive drugs, usually prednisone and azathioprine, which are often combined, with azathioprine playing a steroidsparing role (2,3). However, these drugs may have side effects that can limit or even contraindicate their administration. Among the possible azathioprine-related side effects, interstitial pneumonitis has rarely been reported in adults (4–13), and even more rarely in children (14). The diagnosis must be determined rapidly so that the treatment may be interrupted before irreversible sequelae develop. In such situations, even though corticosteroids are helpful, they may not control the progress of the disease by themselves, making the use of other drugs necessary, among which cyclosporin A appears to be one of the most promising (15). We report on a young girl with autoimmune chronic active hepatitis in whom azathioprine treatment had to be interrupted after the development of interstitial pneumonitis. She was successfully treated with cyclosporin A for 5 years. A relapse occurred a few months later and was controlled with the reintroduction of cyclosporin A.