Felty's syndrome: symptoms, diagnosis

Felty syndrome refers to the complication of rheumatoid arthritis, which occurs only in 1% of all patients with this disease. Fever, muscle atrophy, pigmentation and a tendency to infectious diseases - all these factors usually accompany Felty's syndrome. Diagnosis of pathology is based on the presence in the history of rheumatoid arthritis, the decisive role belongs to laboratory indicators. Treatment involves taking glucocorticosteroids, carrying out splenectomy. For a long time, pathology was characterized by an unclear nature and clinical picture. In 1929, AP Felty described the disease in detail. Since that moment the disease has been differentiated into an independent nosology.

Felty Syndrome: What is it?

The disease is determined by the presence of three pathologies: rheumatoid arthritis, enlarged spleen and leukopenia (low white blood cells). This syndrome is more often diagnosed in people over 40, usually in women.

Experts assume that the underlying cause of the disease is an autoimmune process. It occurs 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 if they occur - in severe course. Therefore, today it is the 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 a disease with 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. It can be a febrile condition, the formation of subcutaneous nodules, myocarditis, weight loss, polyserosites.

Main reasons

The etiology of the disease is unknown. Not all patients with the diagnosis of "rheumatoid arthritis" develop this pathology. In the bone marrow in a healthy person, white blood cells are continuously produced. In patients with this syndrome, in spite of sufficiently low circulation, leukocytes also form. They can accumulate in excess in the spleen.

What are the signs of Felty's syndrome in adults?

• Articular syndrome (polyarthritis of small joints).
• Increased liver size.
• Generalized lymphadenopathy (diagnosed in 50% of cases).
• Progressive increase in the size of the spleen (determined by palpation).
• Polyneuropathy.
• Pigmentation on the skin, the appearance of ulcers in the region of the shins.
• Diffuse pulmonary fibrosis.
• Rheumatoid nodules.

All of the above signs can diagnose Felty's syndrome. Symptoms in all patients manifest themselves in different ways. For example, sometimes the enlargement of the spleen is observed exclusively at 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. The blood test is characterized by anemia, leukopenia, neutropenia. The immunological test usually reveals high titers of the RF, CEC, the presence of antinuclear antibodies. Myelogram results in the diagnosis of myeloid hyperplasia of the brain with a shift toward unripe cellular elements. Instrumental research methods (MRI, CT, ultrasound joints) are often less informative. Felty's syndrome is extremely important to differentiate with liver cirrhosis and sarcoidosis.

Basic principles of treatment

Therapy in this case implies an integrated approach. Rheumatoid arthritis is treated as a standard. Therapy of recurring infections and arthritis includes medicines that help with rheumatism ("Azathioprine", "Methotrexate").

Patients with serious infections are encouraged to do a weekly injection of a factor that stimulates granulocyte colonies. It helps to increase the number of white blood cells.

Basic drugs help to reduce the symptoms that accompanies arthritis and Felty's syndrome itself. These include the following: "Methotrexate", "Penicillamine."

Glucocorticosteroids are effective only in high dosage. Otherwise, the probability of relapse increases. On the other hand, long-term use of glucocorticosteroids may lead to the development of intercurrent infections.

Criteria for the effectiveness of therapy:

• Increase in the number of granulocytes up to 2000 / mm.
• Reduction in the frequency of complications of infectious nature.
• Reducing fever attacks.
• Reduction of manifestations of ulcers on the skin.

When is surgery recommended?

How else can you overcome Felty's syndrome? Treatment through surgical intervention is recommended in the event that the drug therapy was ineffective or because of any reasons it is contraindicated. In this case, speech, as a rule, is about splenectomy. It is important to remember that in 25% of cases, neutropenia recurs after this procedure.

A splenectomy is a surgical operation, during which the doctor completely or partially removes the spleen. Today, there are two possible options for this 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 abdomen, through which he introduces tools for subsequent manipulations. The recovery period, as a rule, does not take much time.

Open splenectomy involves one large incision under the ribs, through which the surgeon removes the spleen. After the removal of this organ, the body's ability to filter out bacteria and viruses from the blood decreases markedly.

Possible complications

Complications of pathology are extremely rare and can occur in the form of rupture of the spleen, the development of 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 very rarely diagnosed today. This does not mean that one can neglect his treatment and do not seek qualified help from a corresponding doctor. Primary symptoms should alert everyone and become a reason to seek advice from a specialist. Otherwise, the likelihood of worsening of the general condition and the development of serious complications, including the rupture of the spleen itself, increases. The only effective treatment option is currently recognized splenectomy.

We hope that all the information on this topic presented in this article will be really useful for you. Be healthy!