Abstract
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.
Highlights
Felty’s syndrome (FS) is a rare clinical syndrome characterized by a triad of seropositive rheumatoid arthritis (RA), with severe joint involvement, splenomegaly, and neutropenia, which occurs in about 1% of RA patients
We reported an FS patient complicated with esophageal gastric variceal bleeding
A series of endoscopic therapy were performed for the control of variceal bleeding successfully, without recurrence of bleeding for two years
Summary
Felty’s syndrome (FS) is a rare clinical syndrome characterized by a triad of seropositive rheumatoid arthritis (RA), with severe joint involvement, splenomegaly, and neutropenia, which occurs in about 1% of RA patients. It was first described in 1924 by the American physician Augustus Roi Felty [1]. Case reports on rituximab and anti-TNFα agents showed promising efficacy. Increased risk of infection and unsatisfactory longterm effects raise concerns for biological agents [3]
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