S5684 A Rare Case of Felty Syndrome-Associated Non-Cirrhotic Portal Hypertension in Sub-Saharan Africa

Introduction: Felty syndrome (FS), an autoimmune disorder that is a subset of seropositive rheumatoid arthritis (RA), is defined by severe RA, idiopathic neutropenia, and splenomegaly. Its estimated prevalence is 1%-3% in the US, and likely lower in Africa. Diagnosis requires chronic idiopathic neutropenia and seropositive RA. Liver involvement is rare but may present as Non-Cirrhotic Portal Hypertension (NCPH), a potentially morbid complication. Case Description/Methods: A 63-year-old woman with no prior autoimmune diagnosis presented to the University Teaching Hospital of Kigali (CHUK) in April 2025 with early satiety, bloating, vomiting, and a chronic left foot wound. She also had chest discomfort, dry cough, night sweats, weakness, and 30 kg unintentional weight loss. Exam showed oral thrush, bilateral joint deformities, a left dorsal foot wound, and stage III splenomegaly. Labs showed severe neutropenia, anemia, mildly elevated ALP, normal transaminases and elevated anti-CCP, ESR/CRP, and rheumatoid factor. Infectious screening was negative. Bone marrow and foot biopsies ruled out dysplasia or malignancy. Chest computed tomography (CT) showed pulmonary fibrosis. Abdominal CT showed hepatosplenomegaly, benign hepatic cysts, portal hypertension without cirrhosis or thrombosis. EGD showed antral gastritis, no varices, ulcers, or bleeding; colonoscopy was unremarkable. She was stabilized and started on empiric antibiotics. On day 5, after positive rheumatologic workup, she was started on oral prednisone, methotrexate, folic acid, and intravenous dexamethasone. By day 8, neutropenia had mildly improved and she was discharged. At 6-week follow-up, she had remained on prednisolone but stopped methotrexate and folic acid. Neutropenia had resolved, splenic size decreased by 0.6 cm, her foot wound had healed, and she had resumed light daily activities. She did not follow up with rheumatology due to limited local access. Discussion: This rare case of FS-associated NCPH in Rwanda highlights the importance of considering systemic autoimmune etiologies in patients presenting with leukopenia and portal hypertension. Though liver biopsy wasn't performed due to limited resources, diagnostic workup supported a diagnosis of FS-associated NCPH. Resolution of neutropenia and clinical improvement following initiation of immunosuppressive therapy further support the diagnosis. Increased awareness of this presentation of NCPH is crucial for timely diagnosis and integrated management, especially in low-resource areas where delayed diagnosis may worsen outcomes.