Felty syndrome is a variant of the course of rheumatoid polyarthritis with significant inhibition of immunity.
Features of development and manifestation of Felty's syndrome.
Felt's syndrome is more common in patients over 40 years of age, usually in women. It is believed that the basis of the development of Felty syndrome is the autoimmune process, which proceeds with the involvement of the lymphoid tissue (lymph nodes and spleen), with the formation of immune complexes, antibodies, oppression of the functional brain and leukocytes. It manifests itself in the form of neutropenia with a decrease in resistance to infections, and when they occur - in their severe course. Therefore, in some cases removal of the spleen is the only effective method of treatment.
The disease is characterized by a large variety, often occurring atypical. In this case, the appearance of Felty syndrome often preceded by several years of the disease of rheumatoid polyarthritis with a predominant lesion of interphalangeal joints of varying degrees of severity.
At the same time, in most patients with Felty's syndrome, there are typical (systemic) manifestations of collagenosis: it may also be subcutaneous nodules, and fever, and muscle atrophy, and myocarditis, and polyserosis, and polyneuropathy, common lymphadenopathy, weight loss.
The most common manifestations of Felty's syndrome are the most common.
- Articular syndrome - polyarthritis of small peripheral joints,
- A progressive increase in the spleen (more than fourfold) - its lower pole may reach the level of the navel and is determined by palpation, but in some cases, the enlargement of the spleen is a late sign,
- Generalized lymphadenopathy occurs in 50%.
- Liver enlargement, occasionally - portal hypertension,
- Dark pigmentation of the open parts of the body, ulcers on the shins,
- Rheumatoid nodules, Sjogren's syndrome,
- Diffuse pulmonary fibrosis and other visceropathies.
They occur rarely and can be manifested in the form of rupture of the spleen, difficult secondary infections, the formation of portal hypertension with gastrointestinal bleeding, development of non-Hodgkin's lymphoma.
Diagnosis of Felty syndrome
- Blood test: decrease in the number of leukocytes, hypochromic anemia, thrombocytopenia, eosinophilia.
- Bone marrow puncture: reducing the number of mature forms of neutrophils, increasing the number of plasma cells.
- Blood serum analysis: the presence of rheumatoid factor, high levels of gamma globulins, transaminases and alkaline phosphatases,
- Ultrasound of the abdominal cavity - enlargement of the size of the liver and spleen (hepatosplenomegaly), portal hypertension, ascites.
Basic Principles for the Treatment of Felt Syndrome
- Glucocorticoids and cytostatics
- For the treatment of rheumatoid polyarthritis in some cases, preparations of gold are shown
- Splenectomy - removal of the spleen with ineffectiveness of conservative therapy.
- Symptomatic therapy and prevention of complications.