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.

中文翻译：

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鼻窦血管疾病是费蒂综合症门脉高压的原因：一例病例报告并文献复习。

Felty's综合征（FS）是一种类风湿关节炎患者发展为脾肿大，嗜中性白血球减少症以及某些情况下门脉高压而无潜在肝硬化的疾病。食管静脉曲张破裂出血是门静脉高压症患者的FS并发症。与脾切除术相反，内镜治疗静脉曲张破裂出血的报道很少。此外，尚未报告长期结果。我们介绍了因Felty综合征继发的门脉高压而导致食管静脉曲张破裂出血的患者。内镜检查后对该患者进行了两年的随访。进行了文献综述并讨论了FS中门脉高压的组织学特征。该患者出现典型的类风湿关节炎（RA），脾肿大，和中性粒细胞减少症，2012年被诊断为Felty综合征。她于2017年9月因食管静脉曲张破裂出血入院。入院时肝功能检查正常。腹部CT显示无肝硬化和门静脉阻塞的迹象。肝活检进一步排除了肝硬化的诊断，并支持了正弦门脉血管疾病（PSVD）的诊断，该疾病以前被称为非肝硬化特发性门静脉高压症（NCIPH）。上腹部内窥镜检查显示胃和食管静脉曲张。进行了一系列内镜检查以结扎食管静脉曲张。对该患者进行了两年的随访，未出现再出血。总之，合并性PSVD可能是FS患者门脉高压的原因。本案在两年内取得了出色的结果，支持使用内窥镜治疗FS患者静脉曲张破裂出血。需要进一步的大范围前瞻性研究来证实研究结果。