Introduction: Gastrointestinal bleeding in rheumatoid arthritis (RA) most commonly occurs from gastroduodenal ulceration in the context of chronic NSAID and prednisone use. We present an unexpected case of variceal bleeding in a patient with RA that was found to be secondary to non-cirrhotic portal hypertension, with liver biopsy revealing nodular regenerative hyperplasia.
Case Description/Methods: A 59-year-old woman with chronic neutropenia and longstanding seropositive RA on prednisone presented with a 3-week history of cough, fevers, hematemesis, and melena. On presentation, she was febrile to 39.4 C and tachycardic with heart rates up to 130 bpm. Physical examination revealed right-sided abdominal tenderness, subcutaneous rheumatoid nodules on her upper extremities, and several spider nevi on the upper chest. Initial laboratory findings were remarkable for a white blood cell count of 16.8x109/L (baseline 2-3x109/L), hemoglobin 4.7 g/dL (baseline 8-9 g/dL), BUN 22 mg/dL, serum albumin 1.5 g/dL, ESR 160 mm/h, and CRP 53 mg/L. She was found to have pneumococcal sepsis, with CT imaging additionally revealing mild splenomegaly measuring 14 cm in length. Evaluation of her hematemesis with upper endoscopy unexpectantly revealed bleeding esophageal varices that were banded. Transjugular liver biopsy revealed nodular regenerative hyperplasia (NRH).In the context of her chronic neutropenia, splenomegaly, and rheumatoid arthritis with further findings of NRH, the patient was diagnosed with Felty Syndrome.
Discussion: Felty Syndrome is a rare extra-articular manifestation of longstanding, joint-deforming, seropositive rheumatoid arthritis defined by the triad of RA, neutropenia, and splenomegaly. A strong association exists between Felty Syndrome and NRH. NRH leads to a form of presinusoidal non-cirrhotic portal hypertension, which may lead to variceal formation and bleeding. This case demonstrates the importance of maintaining a wide differential for upper GI bleeding in patients with RA and highlights the hepatic and gastrointestinal associations in Felty Syndrome.
Figure: CT abdomen and pelvis demonstrating mild splenomegaly
Disclosures:
Rebecca Yao indicated no relevant financial relationships.
Daniela Guerrero Vinsard indicated no relevant financial relationships.
Seth Sweetser indicated no relevant financial relationships.
Rebecca Yao, MD, MPH, Daniela Guerrero Vinsard, MD, Seth Sweetser, MD. C0555 - Non-Cirrhotic Portal Hypertension in a Patient With Rheumatoid Arthritis, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.
