Felty's syndrome refers to a complication of rheumatoid arthritis that occurs in only 1% of all patients with this disease. Fever, muscular atrophy, pigmentation, and a tendency to ailments of an infectious nature - all these factors usually accompany Felty's syndrome. Diagnosis of pathology is based on the presence of a history of rheumatoid arthritis, laboratory parameters are crucial. Treatment involves taking glucocorticosteroids, splenectomy. For a long time, the pathology was distinguished by an unclear nature and a clinical picture. In 1929, A.P. Felty described the disease in detail. From that moment, the disease was differentiated into an independent nosology.
Felty's syndrome: what is it?
The disease is determined by the presence of three pathologies simultaneously: rheumatoid arthritis, an enlarged spleen, and leukopenia (low white blood cell count). This syndrome is more often diagnosed in people over 40 years old, usually in the fair sex.
Experts suggest that the autoimmune process is at the heart of the disease. It proceeds with the direct involvement of lymphoid tissue and the subsequent formation of immune complexes, as well as specific antibodies. Pathology manifests itself in the form of neutropenia with a mandatory decrease in resistance to most infections, and when they occur, in a severe course. Therefore, today it is splenectomy (removal of the spleen) that is recognized as the only effective method of therapy.
Felty's syndrome is very diverse, often atypical. The disease can be preceded by several years (from 5 to about 10) of the disease of the so-called rheumatoid polyarthritis with the simultaneous involvement of interphalangeal joints in the pathological process, with varying degrees of severity.
On the other hand, in most patients with this diagnosis, typical manifestations of collagenosis are determined. This may be a fever, the formation of subcutaneous nodules, myocarditis, weight loss, polyserositis.
Main reasons
The etiology of the disease is unknown. Not all patients diagnosed with rheumatoid arthritis develop this pathology. In a healthy person’s bone marrow, white blood cells are continuously produced. In patients with this syndrome, despite a fairly low circulation, white blood cells also form. They can accumulate in excess in the spleen.
What are the signs of Felty's syndrome in adults?
- Joint syndrome (polyarthritis of small joints).
- An increase in the size of the liver.
- Generalized lymphadenopathy (diagnosed in 50% of cases).
- A progressive increase in the size of the spleen (determined by palpation).
- Polyneuropathy.
- Pigmentation on the skin, the appearance of ulcers in the lower leg.
- Diffuse pulmonary fibrosis.
- Rheumatoid nodules.
All of the above signs can diagnose Felty's syndrome. Symptoms are different for all patients. For example, sometimes an enlargement of the spleen is observed exclusively in the late stages of the development of pathology.
Establishing diagnosis
The basis for the final confirmation of the clinical diagnosis is the simultaneous combination of splenomegaly and rheumatoid polyarthritis. Anemia, leukopenia, neutropenia are characteristic of a blood test. An immunological test usually detects high titers of the Russian Federation, CEC, the presence of antinuclear antibodies. According to the results of a myelogram, myeloid hyperplasia of the brain is diagnosed with a shift towards immature cellular elements. Instrumental methods of research (MRI, CT, ultrasound of the joints) are most often uninformative. Felty's syndrome is extremely important to differentiate with cirrhosis and sarcoidosis.
The basic principles of treatment
Therapy in this case involves an integrated approach. Rheumatoid arthritis is treated as standard. Therapy for recurring infections and arthritis includes drugs that help with rheumatism (Azathioprine, Methotrexate).
Patients with serious infections are advised to inject weekly a factor that stimulates granulocyte colonies. It contributes to an increase in the number of white blood cells.
Basic drugs help reduce the symptoms that accompany arthritis and Felty’s syndrome itself. These include the following: Methotrexate, Penicillamine.
Glucocorticosteroids are effective exclusively in high dosage. Otherwise, the likelihood of relapse increases. On the other hand, prolonged use of glucocorticosteroids can lead to the development of intercurrent infections.
Criteria for the effectiveness of therapy:
- An increase in the number of granulocytes up to 2000 / mm.
- Reducing the frequency of complications of an infectious nature.
- Reduced seizures of fever.
- Reducing the manifestations of ulcers on the skin.
When is surgery recommended?
How else can you overcome Felty's syndrome? Treatment through surgery is recommended if drug therapy is ineffective or is contraindicated for any reason. In this case, it is usually a splenectomy. It is important to remember that in 25% of cases, neutropenia recurs after this procedure.
Splenectomy is a surgical operation during which the doctor completely or partially removes the spleen. Today, two options are possible for carrying out such a procedure: laparoscopy and open splenectomy.
Laparoscopy is performed using general anesthesia. In this case, the operation involves the use of a thin instrument with a camera at the end, which is called a laparoscope. The surgeon makes several small incisions in the abdominal cavity through which he introduces tools for subsequent manipulations. The recovery period, as a rule, does not require much time.
An open splenectomy involves one large incision under the ribs through which the surgeon removes the spleen. After removal of this organ, the body's ability to filter bacteria and viruses from the blood decreases markedly.
Possible complications
Complications of the pathology are extremely rare and can manifest themselves in the form of rupture of the spleen, the development of the so-called portal hypertension with bleeding of the gastrointestinal tract, severe secondary infections.
Conclusion
Felty's syndrome is a relatively rare disease, or rather a complication of a whole group of pathologies, which is rarely diagnosed today. This does not mean that you can neglect his treatment and not seek qualified help from the appropriate doctor. Primary symptoms should alert everyone and become an occasion to seek the advice of a specialist. Otherwise, the likelihood of a deterioration in the general condition and the development of complications that are quite dangerous for health increases, including the rupture of the spleen itself. The only effective treatment option currently recognized is splenectomy.
We hope that all the information on this topic presented in this article will be really useful for you. Be healthy!