■肝结节病是一种罕见的临床疾病,其治疗缺乏明确的建议。我们旨在系统地回顾有关肝结节病治疗的文献,以指导临床医生。
■使用MEDLINE,PubMed,CINAHL,科克伦图书馆,和谷歌学者数据库,我们检索了报告成人肝结节病患者接受各种药物治疗后结局的临床研究的原始文章.主要终点是评估治疗后的症状缓解和生化改善。
■在614个检索到的参考文献中,34项已发表的研究符合资格,提供总共268例肝结节病患者的数据。据报道,仅使用皮质类固醇的一线治疗有187例患者。40例患者使用熊去氧胆酸(UDCA)。在113例(60.4%)和80例(42.8%)的糖皮质激素中,有症状和生化反应。而UDCA显示23例(57.5%)患者完全缓解。二线治疗用于类固醇难治性病例,大多数病例报告为硫唑嘌呤(n=32)和甲氨蝶呤(n=28)。值得注意的是,15例(46.9%)和11例(39.2%)患者分别表现出临床和生化反应。包括抗肿瘤坏死因子(anti-TNF)在内的生物治疗被用作12例患者的三线治疗,症状和生化反应率分别为72.7%。
■肝结节病治疗的证据质量较差。然而,似乎皮质类固醇或UDCA可以用作一线治疗。对于皮质类固醇难治的病例,保留类固醇的免疫抑制剂和抗TNF已显示出一些有希望的结果,但需要进一步的高质量研究。
UNASSIGNED: Hepatic sarcoidosis is an uncommon clinical condition in which clear recommendations are lacking in its treatment. We aimed to review systematically the literature on hepatic sarcoidosis treatment to guide clinicians.
UNASSIGNED: Using MEDLINE, PubMed, CINAHL, Cochrane Library, and Google Scholar databases, we searched original articles on clinical studies reporting the outcome of adult hepatic sarcoidosis patients following treatment with various pharmacological agents. The primary end point was focused on assessing symptomatic relief and biochemical improvement posttreatment.
UNASSIGNED: Out of 614 retrieved references, 34 published studies were eligible, providing data for a total of 268 patients with hepatic sarcoidosis. First-line therapy with corticosteroids alone was reported in 187 patients, whilst ursodeoxycholic acid (UDCA) was used in 40 patients. Symptomatic and biochemical responses were reported among 113(60.4%) and 80(42.8%) cases of corticosteroids respectively, whereas UDCA showed a complete response in 23(57.5%) patients. Second-line therapy was used in steroid-refractory cases, with most cases being reported for azathioprine (n = 32) and methotrexate (n = 28). Notably, 15(46.9%) and 11(39.2%) patients showed both clinical and biochemical responses respectively. Biological therapy including anti-tumor necrosis factor (anti-TNF) was used as third line therapy in twelve cases with a 72.7% symptomatic and biochemical response rate each.
UNASSIGNED: The quality of evidence for the treatment of hepatic sarcoidosis was poor. Nevertheless, it appears that corticosteroid or UDCA may be utilized as first-line therapy. For cases that are refractory to corticosteroids, steroid-sparing immunosuppressive agents and anti-TNF have shown some promising results, but further high-quality studies are required.