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肝结节病:病例系列

Hepatic sarcoidosis: a case series.

作者信息

Ennaifer Rym, Ayadi Shema, Romdhane Hayfa, Cheikh Myriam, Nejma Houda Ben, Bougassas Wassila, Hadj Najet Bel

机构信息

Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia.

出版信息

Pan Afr Med J. 2016 Jul 8;24:209. doi: 10.11604/pamj.2016.24.209.7980. eCollection 2016.

Abstract

Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.

摘要

结节病是一种全身性非干酪样肉芽肿疾病。肝脏是常见受累部位,但通常无症状。目前缺乏针对该部位治疗的循证指南,且皮质类固醇的疗效可能欠佳。我们研究的目的是描述7例系统性结节病合并肝脏受累患者的临床、生化、放射学及治疗特征。这是一项为期3年的回顾性单中心描述性研究,纳入了7例系统性结节病合并肝脏受累的患者。我们纳入了5名女性和2名男性,平均年龄43岁。肝脏定位检查发现5例存在结节病。所有患者均观察到肝肿大,以及以无黄疸型胆汁淤积为表现的血清肝功能异常。肝活检显示所有病例均有符合结节病的肉芽肿性病变,2例有严重纤维化。所有患者均有肝外表现,主要为肺部受累。所有患者均接受了治疗,5例使用皮质类固醇,2例使用熊去氧胆酸(UDCA)。1例观察到完全缓解,另1例部分缓解,1例对皮质类固醇耐药。2例患者使用皮质类固醇治疗不足1个月,无法评估疗效。1例对类固醇耐药的肝病患者联合使用抗疟药和UDCA取得成功。肝脏受累可能提示系统性结节病。鉴于有进展为严重肝病的风险,所有系统性结节病患者均须进行筛查。治疗并非常规进行,仍以皮质类固醇治疗为主。在缺乏前瞻性随机对照试验的情况下,UDCA的疗效有待证实。

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