Felty's syndrome
Encyclopedia
Felty's syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid arthritis
, splenomegaly
and neutropenia
.
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).
Splenectomy
may improve neutropenia
in severe disease.
Use of rituximab
and leflunomide
have been proposed.
Use of gold
therapy has also been described.
Rheumatoid arthritis
Rheumatoid arthritis is a chronic, systemic inflammatory disorder that may affect many tissues and organs, but principally attacks synovial joints. The process produces an inflammatory response of the synovium secondary to hyperplasia of synovial cells, excess synovial fluid, and the development...
, splenomegaly
Splenomegaly
Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any...
and neutropenia
Neutropenia
Neutropenia, from Latin prefix neutro- and Greek suffix -πενία , is a granulocyte disorder characterized by an abnormally low number of neutrophils, the most important type of white blood cell...
.
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
Spleen
The spleen is an organ found in virtually all vertebrate animals with important roles in regard to red blood cells and the immune system. In humans, it is located in the left upper quadrant of the abdomen. It removes old red blood cells and holds a reserve of blood in case of hemorrhagic shock...
(splenomegaly) and abnormally low levels of certain white blood cells
(neutropenia
Neutropenia
Neutropenia, from Latin prefix neutro- and Greek suffix -πενία , is a granulocyte disorder characterized by an abnormally low number of neutrophils, the most important type of white blood cell...
). 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).
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.Complications
- Recurrent Infection,
- Hypersplenism causing anaemia and thrombocytopeniaThrombocytopeniaThrombocytopenia is a relative decrease of platelets in blood.A normal human platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. These limits are determined by the 2.5th lower and upper percentile, so values outside this range do not necessarily indicate disease...
- LymphadenopathyLymphadenopathyLymphadenopathy is a term meaning "disease of the lymph nodes." It is, however, almost synonymously used with "swollen/enlarged lymph nodes". It could be due to infection, auto-immune disease, or malignancy....
- Skin hyperpigmentationHyperpigmentationIn dermatology, hyperpigmentation is the darkening of an area of skin or nails caused by increased melanin.-Causes:Hyperpigmentation may be caused by sun damage, inflammation, or other skin injuries, including those related to acne vulgaris...
& 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. It affects less than 1% of patients with rheumatoid arthritis.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.Splenectomy
Splenectomy
A splenectomy is a surgical procedure that partially or completely removes the spleen.-Indications:The spleen, similar in structure to a large lymph node, acts as a blood filter. Current knowledge of its purpose includes the removal of old red blood cells and platelets, and the detection and fight...
may improve neutropenia
Neutropenia
Neutropenia, from Latin prefix neutro- and Greek suffix -πενία , is a granulocyte disorder characterized by an abnormally low number of neutrophils, the most important type of white blood cell...
in severe disease.
Use of rituximab
Rituximab
Rituximab, sold under the trade names Rituxan and MabThera, is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells...
and leflunomide
Leflunomide
Leflunomide is a medication of the DMARD type, used in active moderate to severe rheumatoid arthritis and psoriatic arthritis. It is a pyrimidine synthesis inhibitor.-Basic chemical, pharmacological, and marketing data:...
have been proposed.
Use of gold
Gold
Gold is a chemical element with the symbol Au and an atomic number of 79. Gold is a dense, soft, shiny, malleable and ductile metal. Pure gold has a bright yellow color and luster traditionally considered attractive, which it maintains without oxidizing in air or water. Chemically, gold is a...
therapy has also been described.
See also
- Still's diseaseStill's diseaseStill's disease can refer to:* Juvenile idiopathic arthritis* Adult-onset Still's disease...
or Still-Chauffard-Felty syndrome, the juvenile form - Caplan's SyndromeCaplan's syndromeCaplan's syndrome is a combination of rheumatoid arthritis and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray.. The syndrome is named after Dr...