Felty syndrome: causes, symptoms, treatment, prevention, complications
Felty syndrome is a complication on the background of rheumatoid arthritis
Complicated by rheumatoid arthritis can lead to the syndrome Felty. Three pathology may indicate the emergence of the disease: diagnosis of rheumatoid arthritis, significantly reduced white blood cells, physical enlargement of the spleen.
Consider the syndrome is diagnosed very rarely. Studies show that only 1 out of 100 patients will develop Felty syndrome.
The results showed that 70% of those affected are women over the age of 40-50 years. Therefore, in rheumatology, the disease is extremely rare, there are many gaps in the diagnosis and treatment of which physicians are trying to fill through research.
In children, this syndrome is diagnosed less frequently than in adults. His descriptions in the medical literature are given in the order of casuistry. Since 1929, when Felty made a detailed description of the combination of three main symptoms, were not made significant additions.
The disease somewhat resembles articular-visceral rheumatoid arthritis. Already at the initial stage of the disease is diagnosed with significant enlargement of the spleen. In the course of the disease in the acute form it can reach significant sizes.
Syndrome attributed to associated diseases. Enlargement of the spleen is due to circulating immune complexes, which cause dysfunction of neutrophils. Confirmation of the above statements is getting detected in the cytoplasm of neutrophils of patients with deposits of antibodies.
The etiology and pathogenesis of violations
As previously noted, the disease is not fully understood, and the causes of this development are found: in some patients with rheumatoid arthritis it can appear and some don’t. It is only known how the appearance of the syndrome.
The process of the emergence of Felty syndrome can be described as follows:
- in the bone marrow the formation of white blood cells;
- such education occurs as they propagate with a small velocity;
- due to the excessive number and small circulation occurs, their accumulation in the spleen.
Patients who have antibody syndrome is diagnosed quite often. Antibodies fight against blood cells, accumulation alone leads to a significant increase in the other.
Pathogenesis (what is happening in the body when you syndrome) can be characterized by a decrease in the expression of HLA-DRw4 in the formation of antibodies. Many doctors explain it by hypersplenism. However, there are some deviations: the ongoing splenectomy does not cure the patient.
Characteristics of the clinical picture
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Direct symptoms of the disease not. An example is the fact that in some patients infection (skin type, or pneumonia) occur more frequently than in a healthy person.
This is due to the low content of lymphocytes. The body becomes vulnerable to inflammatory processes, which leads to the appearance of the infection. In severe cases ulcers develop on the feet.
The symptoms by which to diagnose Felty Syndrome, the following:
- the offensive in the early period of disease development of leukopenia;
- develops thrombocytopenia and granulocytopenia, and anemia;
- destroyed and neutrophils that accumulate in the spleen.
After a study was conducted of the spleen it is possible to identify hyperplasia of the pulp. Vessels can be filled blood and greatly expanded, find large areas of necrosis. Necrosis occurs due to vasculitis, the occurrence of which is typical in the development of rheumatoid arthritis.
If the symptoms have not been identified at an early stage, further examination of the bone marrow reveals a violation of the maturation of the main elements involved in the formation of neutrophils.
In turn, disruption of the bone marrow will cause a reduction in the concentration of neutrophils in Mature form. The decrease in the concentration of neutrophils is the cause of a large number of plasma cells.
Swollen lymph nodes can indicate different diseases, but also occurs (in small or large extent) in the Felty syndrome. Secondary symptoms include:
- rapid loss of body weight under normal diet;
- the formation of pigmentation on the skin;
- change the size of the liver;
- necrotic stomatitis;
Secondary symptoms development syndrome can be any infectious disease. It is important to pay attention to their frequency of occurrence and should be tested for lymphocytes.
Diagnosis and treatment: challenges and opportunities
Unfortunately, due to the fact that the reasons for the development of the syndrome have not been identified, the exact method of determining its development there. Only an experienced doctor when comparing the three conditions and obtained results of assigned research may suggest about the development of this rare disease.
Pay special attention to Felty syndrome with the rapid development of rheumatoid arthritis. Conducted blood tests can detect a large number of antibodies are formed to fight white blood cells.
In some cases, to take any actions. All doctor appointments are only associated with the treatment of rheumatoid arthritis, which has the standard form: prescription of Methotrexate or Azathioprine.
Also undergoing treatment for other symptoms and diseases, developing on the background of arthritis and Felty syndrome. If that appears, the infection can bring severe damage to health, administered a special injection to stimulate the production of blood cells. In advanced cases, is removal of the spleen.
Effective drugs that can reduce the clinical manifestation of arthritis include:
- Gold salts.
Corticosteroids have efficacy only at high doses. Examples would include receiving Methotrexate. At lower doses there is often a relapse. However the prolonged use of the drug in large doses increases the risk of intercurrent infections, as GK is not physiological.
When surgery is needed
Splenectomy – the surgical procedure performed for complete or partial removal of the spleen. As previously noted, only in rare cases, doctors prescribe surgery.
Often such a purpose is due to the ineffectiveness of conservative treatment. Splenectomy is also not an effective means of dealing with atropia, as in 25% of cases experience a recurrence almost immediately after surgery.
After carrying out the intervention under consideration frequency of intercurrent infections is not reduced. That’s why doctors only in extreme cases carry out the purpose of splenectomy.
Despite the rarity of the disease, ongoing research aimed at finding more effective treatment of Felty syndrome.
Examples would include the appearance of drugs based on lithium which stimulate granulocytopoiesis. However, new drugs have quite a lot of side effects. The main thing to exclude is the development of infections that can greatly worsen the patient’s condition.
Forecast and consequences
The spleen is responsible for the formation of the immune system.
Complication of development of considered syndrome can be an increase in the frequency of emergence of infectious diseases.
Also do not forget about increasing the size of the body, it also causes a lot of problems.
To more serious consequences include:
- ruptured spleen due to a significant increase of its size;
- intercurrent infectious diseases;
- hypertension with concomitant gastrointestinal bleeding.
Complications threaten the patient’s life and health. Therefore, the physician should not idle when identifying considered a rare disease.
Diagnose Felty syndrome may not in many clinics due to the lack of an efficient method of its determination. Therefore, when an increased risk (age over 40 years, the development of rheumatoid arthritis) should be treated in private clinics with a good reputation.