Ebert Ellen C, Kierson Malca, Hagspiel Klaus D
Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, New Jersey 08903, USA.
Am J Gastroenterol. 2008 Dec;103(12):3184-92; quiz 3193. doi: 10.1111/j.1572-0241.2008.02202.x. Epub 2008 Oct 1.
Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
结节病是一种多系统疾病,其特征是在包括皮肤、心脏、神经系统和关节等受累器官中出现非干酪样肉芽肿。结节病的诊断通常基于相符的病史、至少两个不同器官中肉芽肿的显示、抗酸杆菌染色和培养阴性、无职业或家庭毒素暴露以及无药物诱发疾病。中空器官受累罕见。一些报道的黏膜病变可能并非由结节病引起,而只是偶然出现肉芽肿。淋巴结病的外在压迫可发生于整个胃肠道。胃,尤其是胃窦,是最常受累的肝外器官,而小肠是最不常受累的。肝脏受累很常见,范围从无症状的偶然肉芽肿到门静脉三联征肉芽肿导致的门静脉高压,通常肝功能相对保留。CT扫描显示肝脾肿大和淋巴结病,其次常见的是肝脏和脾脏的局灶性低密度病变。腹水通常是右心衰竭(由于肺动脉高压)或门静脉高压(由于胆汁性肝硬化)导致的漏出液。很少情况下,由于腹膜上有结节可出现渗出性腹水。胰腺受累表现为肿块,通常在胰头或表现为弥漫性坚实的结节状器官。当器官功能受到威胁时,通常是肺、眼和中枢神经系统,应使用皮质类固醇。它们在肝结节病治疗中的作用尚不清楚。尽管大多数患者会有一些永久性器官损害,但总体预后良好。心脏和肺部疾病是主要死因。