Center of Liver Diseases, Beijing Ditan Hospital of Capital Medical University, Beijing, China.
Division of Gastroenterology and Hepatology, Department of Medicine, NYU Langone Health, New York University School of Medicine, New York 11355, USA.
Biomed Res Int. 2020 Jul 1;2020:2618260. doi: 10.1155/2020/2618260. eCollection 2020.
Felty's syndrome (FS) is a disorder wherein patients with rheumatoid arthritis develop splenomegaly, neutropenia, and in some cases, portal hypertension without underlying cirrhosis. Esophageal variceal bleeding is a complication of FS in patients with portal hypertension. In contrast to splenectomy, few reports exist on the management of variceal bleeding with endoscopic therapy. Moreover, the long-term outcome has not been reported. We present a patient with esophageal variceal bleeding due to portal hypertension secondary to Felty's syndrome. The patient was followed up for two years postendoscopy intervention. Literature review was performed and the histological features of portal hypertension in FS are discussed. The patient presented with a typical triad of rheumatoid arthritis (RA), splenomegaly, and neutropenia and was diagnosed as Felty's syndrome in 2012. She was admitted to our hospital in September 2017 for esophageal variceal bleeding. At the time of admission, her liver function test was normal. Abdominal CT showed no signs of cirrhosis and portal vein obstruction. Liver biopsy further excluded diagnosis of cirrhosis and supported the diagnosis of porto-sinusoidal vascular disease (PSVD), which was previously named as noncirrhotic idiopathic portal hypertension (NCIPH). An upper abdominal endoscopy revealed gastric and esophageal varices. A series of endoscopies was performed to ligate the esophageal varices. The patient was followed up for two years and did not show rebleeding. In conclusion, comorbid PSVD might be a cause of portal hypertension in FS patients. The present case had excellent outcome in two years, which supported the use of endoscopic therapy for the management of variceal bleeding in FS patients. Further large prospective study is needed to confirm the findings.
费尔蒂综合征（FS）是一种病症，患有类风湿性关节炎的患者会出现脾肿大、中性粒细胞减少症，在某些情况下还会出现门静脉高压，而没有潜在的肝硬化。食管静脉曲张出血是 FS 患者门静脉高压的并发症。与脾切除术不同，很少有关于内镜治疗静脉曲张出血管理的报告。此外，长期结果尚未报道。我们报告了一例继发于费尔蒂综合征的门静脉高压性食管静脉曲张出血患者。该患者在内镜治疗干预后进行了两年的随访。我们进行了文献复习，并讨论了 FS 中门静脉高压的组织学特征。该患者表现为典型的类风湿关节炎（RA）三联征、脾肿大和中性粒细胞减少症，并于 2012 年被诊断为费尔蒂综合征。她于 2017 年 9 月因食管静脉曲张出血入住我院。入院时，肝功能检查正常。腹部 CT 未显示肝硬化和门静脉阻塞的迹象。肝脏活检进一步排除了肝硬化的诊断，并支持了门腔静脉血管疾病（PSVD）的诊断，PSVD 以前被称为非肝硬化特发性门静脉高压症（NCIPH）。上腹部内镜检查显示胃和食管静脉曲张。进行了一系列内镜套扎术以结扎食管静脉曲张。该患者随访两年，未再出现出血。总之，合并 PSVD 可能是 FS 患者门静脉高压的一个原因。本病例在两年内有良好的预后，支持内镜治疗 FS 患者静脉曲张出血的管理。需要进一步的大型前瞻性研究来证实这些发现。
