Felty's syndrome

Felty's syndrome, also called Felty syndrome,^[1] is characterized by the combination of rheumatoid arthritis, splenomegaly and neutropenia. The condition is more common in those aged 50–70 years, and is more prevalent in females than males and more in Caucasians than those of African descent. It is a deforming but inactive disease and seropositive for RF.^[2][3]

Symptoms

The symptoms of Felty's syndrome are similar to those of rheumatoid arthritis. Patients suffer from painful, stiff, and swollen joints, most commonly in the joints of the hands, feet, and arms. In some affected individuals, Felty's syndrome may develop during a period when the symptoms and physical findings associated with rheumatoid arthritis have subsided or are not present. In this case, Felty's syndrome may remain undiagnosed. In more rare instances, the development of Felty's syndrome may precede the development of the symptoms and physical findings associated with rheumatoid arthritis.

Felty's syndrome is also characterized by an abnormally enlarged spleen (splenomegaly) and abnormally low levels of certain white blood cells (neutropenia). As a result of neutropenia, affected individuals are increasingly susceptible to certain infections.In some patients keratoconjuntivitis sicca due to secondary sjogren syndrome is seen.

Individuals with Felty's syndrome may also experience fever, weight loss, and/or fatigue. In some cases, affected individuals may have discoloration of the skin, particularly of the leg (abnormal brown pigmentation), sores (ulcers) on the lower leg, and/or an abnormally large liver (hepatomegaly). In addition, affected individuals may have abnormally low levels of circulating red blood cells (anemia), a decrease in circulating blood platelets that assist in blood clotting functions (thrombocytopenia), abnormal liver function tests and/or inflammation of the blood vessels (vasculitis).^[4]

Causes

The cause of Felty's syndrome is unknown. It is more common in people who have had rheumatoid arthritis for a long time. Some patients have Human Leucocytic Antigen (HLA-DR4) in their serum. This syndrome is mostly present in people having extra articular manifestations of rheumatoid arthritis. People with this syndrome are at risk of infection because they have a low white blood cell count.^[5]

Complications

• Recurrent Infection,
• Hypersplenism causing anemia and thrombocytopenia
• Lymphadenopathy
• Skin hyperpigmentation & cutaneous ulceration

Diagnosis

Felty's syndrome is defined by the presence of three conditions: rheumatoid arthritis, an enlarged spleen (splenomegaly), and an abnormally low white blood count. Anaemia(normochromic,normocytic), thrombocytopenia, neutropenia, abnormal liver function tests are other lab findings. It affects less than 1% of patients with rheumatoid arthritis.^[6]

Treatment

The best treatment for Felty's syndrome is to control the underlying RA. Immunosuppressive therapy for RA often improves granulocytopenia and splenomegaly; this finding reflects the fact that Felty's syndrome is an immune-mediated disease. Most of the traditional medications used to treat RA have been used in the treatment of Felty's syndrome. No well-conducted, randomized, controlled trials support the use of any single agent. Most reports on treatment regimens involve small numbers of patients.^[7]

Splenectomy may improve neutropenia in severe disease.

Use of rituximab^[8] and leflunomide^[9] have been proposed.

Use of gold therapy has also been described.^[10][11]

Eponym

It is named for Augustus Roi Felty.^[12][13]

See also

• Caplan's syndrome
• Still's disease or Still-Chauffard-Felty syndrome, the juvenile form

References