Felty syndrome is a rare complication of rheumatoid arthritis (RA) that affects a small percentage of individuals with this autoimmune disease. It is characterized by the triad of rheumatoid arthritis, an enlarged spleen (splenomegaly), and a low white blood cell count (neutropenia). The exact cause of Felty syndrome is unknown, but it is believed to be related to the chronic inflammation associated with RA.
Rheumatoid Arthritis: The presence of Felty syndrome requires a diagnosis of rheumatoid arthritis. RA is a chronic autoimmune disorder that primarily affects the joints, causing pain, swelling, stiffness, and reduced mobility. Individuals with Felty syndrome often have severe and long-standing RA.
Splenomegaly: Enlargement of the spleen is a hallmark feature of Felty syndrome. The spleen is an organ located in the upper left side of the abdomen, and its main function is to filter the blood and remove old or damaged red blood cells. In Felty syndrome, the spleen becomes enlarged due to the accumulation of abnormal white blood cells and increased blood flow. Splenomegaly may cause discomfort or pain in the left upper abdomen.
Neutropenia: Neutropenia refers to a low white blood cell count, specifically a decrease in neutrophils, which are a type of white blood cell that plays a crucial role in fighting off infections. In Felty syndrome, the neutrophil count is significantly reduced, making individuals more susceptible to recurrent infections, particularly in the skin, respiratory tract, and urinary tract. These infections may be severe and difficult to treat.
Other Symptoms: In addition to the triad of RA, splenomegaly, and neutropenia, individuals with Felty syndrome may experience other symptoms, including:
Complications: Felty syndrome can lead to various complications due to the increased risk of infections and the presence of chronic inflammation. These complications may include skin infections, lung infections, septicemia (bloodstream infection), and an increased risk of lymphoma.
Treatment: The management of Felty syndrome focuses on controlling the symptoms, reducing inflammation, and preventing infections. Treatment options may include disease-modifying antirheumatic drugs (DMARDs) to manage RA, immunosuppressive medications to suppress the immune system, and medications to stimulate white blood cell production. In severe cases, splenectomy (surgical removal of the spleen) may be considered to improve symptoms and reduce the risk of infections.