The cause remains unknown. However, it is more common in RA patients (approximately 1%-3%) after living with RA for a decade or more.
FS is most prevalent in individuals aged 50-70 years of age and is rare in children. There is a female predisposition. FS usually occurs in patients of Northern European descent; it is less common in patients of African descent.
Clinical signs and symptoms include splenomegaly, hepatomegaly, lymphadenopathy, malaise, fatigue, anorexia, weight loss, pallor, rheumatoid nodules, joint deformities, synovitis, recurring infections, and ocular burning or discharge.
The most significant part of treatment is controlling the underlying RA. Immunosuppressive therapy for RA treatment usually improves granulocytopenia and splenomegaly. Splenectomy may be suggested in some cases. FS patients with serious, potentially life-threatening infections should be admitted to the hospital and closely monitored.
Even though many FS patients are asymptomatic, some may develop severe infections secondary to granulocytopenia. Infections of the skin and lung are common. Prognosis is highly dependent on the level of severity of the underlying RA, in combination with the use of immunosuppressive therapy used in RA and FS treatment.
For more information, see OMIM.
M05.00 – Felty's syndrome, unspecified site
57160007 – Felty's syndrome
Last Updated: 03/29/2017