Felty's syndrome

Felty's syndrome is a disorder characterized by the combination of rheumatoid arthritis, splenomegaly and neutropenia.^[1]

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. It affects less than 1% of patients with rheumatoid arthritis.^[2]

Causes

The cause of Felty's syndrome is unknown. It is more common in people who have had rheumatoid arthritis for a long time. People with this syndrome are at risk of infection because they have a low white blood cell count.^[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.

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), and/or inflammation of the blood vessels (vasculitis).^[4]

Complications

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

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.^[5]

Splenectomy may improve neutropenia in severe disease.

Use of rituximab^[6] and leflunomide^[7] have been proposed.

Eponym

It is named for A. R. Felty.^[8][9]

See also

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

References