Devaney K, Goodman Z D, Epstein M S, Zimmerman H J, Ishak K G
Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, D.C.
Am J Surg Pathol. 1993 Dec;17(12):1272-80.
The patterns of hepatic injury were studied in 100 patients with a diagnosis of sarcoidosis and clinical evidence of liver disease that led to diagnostic liver biopsy. Granulomas were present in all patients; they occupied from < 1% to > 90% of the total volume of tissue examined and were most often located in the portal/periportal region. In none of the 100 cases were infectious organisms identified by special stains, culture, or serology. In 99% of cases, these granulomas were noncaseating; in one of the 100 cases central caseation was noted. In addition to the granulomas present in all biopsies, three broad categories of histologic change were found: cholestatic (58%), necroinflammatory (41%), and vascular (20%). Among those with cholestasis, 19 patients had bile duct lesions similar to primary biliary cirrhosis, whereas another 13 had a pattern of periductal fibrosis reminiscent of primary sclerosing cholangitis. In 37 patients with chronic cholestasis, a decrease in the number of bile ducts (ductopenia) was noted. Twelve patients had an acute cholangitis suggestive of mechanical obstruction--although no clinical evidence of ductal obstruction was found. Necroinflammatory changes included spotty necrosis suggesting hepatitis of diverse etiologies (including viral infection and drug reaction) and chronic portal inflammation suggestive of chronic active hepatitis. Vascular changes consisted of sinusoidal dilatation (14 cases) and nodular regenerative hyperplasia (9 cases). In 6% of the patients, the only changes in the biopsy were those of granulomatous inflammation; each of these patients had a dominant mass ("sarcoidoma"), which had been biopsied to rule out tumor. Fibrosis was seen in 21% of the biopsies--periportal (13%), bridging (2%), or cirrhosis (6%). It is clear that sarcoidosis can cause progressive liver disease with a wide array of histologic features that can mimic those of other primary liver diseases.
对100例诊断为结节病且有肝脏疾病临床证据并因此接受诊断性肝活检的患者的肝损伤模式进行了研究。所有患者均存在肉芽肿；肉芽肿占所检查组织总体积的比例从<1%到>90%不等，且最常位于门静脉/门静脉周围区域。在这100例病例中，通过特殊染色、培养或血清学均未发现感染性生物体。在99%的病例中，这些肉芽肿为非干酪样；100例中有1例出现中央干酪样变。除所有活检中均存在的肉芽肿外，还发现了三大类组织学改变：胆汁淤积性（58%）、坏死性炎症性（41%）和血管性（20%）。在胆汁淤积患者中，19例有类似于原发性胆汁性肝硬化的胆管病变，而另外13例有类似于原发性硬化性胆管炎的导管周围纤维化模式。在37例慢性胆汁淤积患者中，发现胆管数量减少（胆管减少）。12例患者有提示机械性梗阻的急性胆管炎——尽管未发现导管梗阻的临床证据。坏死性炎症性改变包括提示多种病因肝炎（包括病毒感染和药物反应）的点状坏死以及提示慢性活动性肝炎的慢性门静脉炎症。血管性改变包括窦性扩张（14例）和结节状再生性增生（9例）。6%的患者活检中唯一的改变是肉芽肿性炎症；这些患者均有一个主要肿块（“结节瘤”），已对其进行活检以排除肿瘤。21%的活检中可见纤维化——门静脉周围（13%）、桥接（2%）或肝硬化（6%）。显然，结节病可导致具有多种组织学特征的进行性肝病，这些特征可模仿其他原发性肝病的特征。
