Review Question - QID 220358

The patient is presenting with signs of rheumatoid arthritis, splenomegaly, and neutropenia. These findings are characteristic of Felty syndrome.

Rheumatoid arthritis can be associated with multiple extraarticular syndromes such as Felty syndrome. This syndrome is characterized by a triad of rheumatoid arthritis, neutropenia, and splenomegaly. Symptoms of rheumatoid arthritis include fatigue, morning stiffness that lasts over 1 hour, arthritis of the MCP and PIP joints that can progress to ulnar deviation of the fingers or subluxation at the MCP joints, and possibly rheumatoid nodules. Lab testing in Felty syndrome will show an absolute neutrophil count of less than 2000/µL. Labs may also show anemia (which is due to anemia of chronic disease) and splenomegaly on exam. Treatment includes methotrexate and other disease-modifying anti-rheumatic drugs like tumor necrosis factor inhibitors and hydroxychloroquine.

Klein and Molad review the evidence regarding the diagnosis of hematological manifestations in rheumatic diseases. They discuss how Felty syndrome can be seen in patients with rheumatoid arthritis. They recommend considering these diseases so that they are not overlooked.

Figure/Illustration A is a radiograph of the hand demonstrating subluxation of the metacarpophalangeal joints (red circles). This finding is classically seen in patients with rheumatoid arthritis.

Incorrect Answers:
Answer 1: Caplan syndrome is a combination of seropositive rheumatoid arthritis and pneumoconiosis. Pneumoconiosis presents as cough, shortness of breath, and characteristic radiographic chest findings such as rounded opacities of varying sizes. It is believed that patients have an immune hypersensitivity to foreign bodies in the lungs, which triggers genetically predisposed individuals to develop autoimmunity like rheumatoid arthritis.

Answer 2: Epstein-Barr virus causes mononucleosis, which can present as fatigue, fever, tonsillar exudates, posterior cervical lymphadenopathy, and hepatosplenomegaly. Labs can show mild neutropenia, and a peripheral smear will show atypical lymphocytes. This disease would not explain the patient’s radiographic joint findings, and this patient does not have pharyngitis.

Answer 4: Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas. Patients can present with dyspnea, subcutaneous nodules or papules, and adenopathy. Sarcoidosis can have osteoarticular involvement, but it is uncommon and will usually have associated lung involvement. Treatment may include steroids.

Answer 5: Sickle cell disease is a disorder of abnormal hemoglobin. It can cause vaso-occlusive crises that can present either as chest pain, bone pain, fever, dactylitis, stroke, or abdominal pain due to gallstones or splenomegaly. Labs will show anemia and a high reticulocyte count, and a peripheral blood smear will show sickling of red blood cells. Sickle cell disease would not explain the patient’s radiographic finding of MCP joint subluxation.

Bullet Summary:
Felty syndrome presents in patients with a triad of rheumatoid arthritis, neutropenia, and splenomegaly.