Amjad Waseem, Jagroop Sophia, Parthvi Rukma
Forest Hills Hospital, Northshore-Long Island Jewish Health System.
Cureus. 2017 Jan 25;9(1):e996. doi: 10.7759/cureus.996.
Both polycystic liver disease (PLD) and sarcoidosis can involve liver. Most of the time, liver disease in both conditions is asymptomatic, but they can rarely cause portal hypertension. Our aim is to report a case of a 51-year-old female with a history of adult dominant polycystic kidney disease (ADPKD) and sarcoidosis who presented with multiple episodes of hematemesis. An endoscopy showed grade 3 esophageal varices. A computed tomography (CT) scan of the abdomen showed ascites with polycystic liver, nodular contour, and calcified granuloma. PLD can cause portal hypertension due to fibrosis or large cysts compressing on the portal vein. On the other hand, sarcoidosis causes portal hypertension by formation of arteriovenous(AV) shunts or fibrosis in areas of granulomas. Both conditions are diagnosed on imaging. There is no approved medical treatment for PLD; the only curative treatment is liver transplantation. Asymptomatic hepatic sarcoidosis does not need any treatment. The recommended treatment is corticosteroids for both isolated and systemic sarcoidosis. ADPKD and sarcoidosis can involve multiple organs. The presence of both conditions can accelerate the disease process and could be a therapeutic challenge. Early abdominal imaging during the course of both diseases can improve the outcome by decreasing the diagnostic window.
多囊肝病（PLD）和结节病均可累及肝脏。大多数情况下，这两种疾病所致的肝脏病变均无症状，但它们极少会引起门静脉高压。我们旨在报告一例51岁女性患者，该患者有成人显性多囊肾病（ADPKD）和结节病病史，曾多次出现呕血。内镜检查显示为3级食管静脉曲张。腹部计算机断层扫描（CT）显示腹水伴多囊肝、结节状轮廓和钙化肉芽肿。PLD可因纤维化或大囊肿压迫门静脉而导致门静脉高压。另一方面，结节病通过在肉芽肿区域形成动静脉（AV）分流或纤维化而导致门静脉高压。这两种疾病均通过影像学诊断。目前尚无获批用于治疗PLD的药物；唯一的治愈性治疗方法是肝移植。无症状性肝结节病无需任何治疗。对于孤立性和全身性结节病，推荐的治疗方法是使用皮质类固醇。ADPKD和结节病可累及多个器官。这两种疾病并存可加速疾病进程，并且可能是一个治疗难题。在这两种疾病的病程中尽早进行腹部影像学检查，可通过缩短诊断时间窗来改善治疗效果。
