Felty syndrome is a disease of the rheumatic type. The inflammatory rheumatic disease is considered a special form of the so-called rheumatoid arthritis. In 1924, Felty syndrome was described for the first time.
What is Felty syndrome
Infographic of pain regions and affected joints in rheumatoid arthritis. Click image to enlarge. Felty syndrome affects women far more often than men. The peak age of onset in women is between the ages of 45 and 50, and 65 and 70. Rheumatism experts classify Felty’s syndrome diagnostically as a special form of polyarthritis. Before patients are diagnosed with Felty’s syndrome, they have already had rheumatoid arthritis. Not every affected patient develops the clinical signs of Felty syndrome over time. On average, more than 12 years elapse between the initial manifestation of polyarthritis and the diagnosis of Felty syndrome. To make the diagnosis of Felty syndrome, the clinical manifestation of a so-called triad is mandatory. In Felty’s syndrome, affected individuals regularly suffer from severe rheumatic pain and limitation of movement. Destructions and deformities at the joints or joint ends can assume considerable proportions.
Causes
As with most rheumatic diseases, the causes of invasive inflammatory joint changes in Felty syndrome are still largely obscure. Since Felty’s syndrome, in contrast to rheumatoid arthritis, is a rare rheumatoid variant, there is a lack of public funding, according to the German Rheumatism League, which would, however, be urgently needed for research into the causes. Felty’s syndrome occurs in less than 1 percent of cases of so-called uncomplicated rheumatoid arthritis. If vessels are also affected by the rheumatic-inflammatory change, physicians speak of vasculitis. Patients with vasculitides are affected by Felty’s syndrome in about 7 percent of cases. With advancing age, the probability of developing Felty’s syndrome increases for patients with rheumatoid arthritis. Recent research suggests that Felty syndrome may be an autoimmune disease, meaning that the organism produces antibodies against the body’s own cartilage and bone structures, which in turn results in an inflammatory response.
Symptoms, complaints, and signs
Felty syndrome is associated with very unpleasant symptoms, all of which have a very negative impact on the quality of life of the affected person. As a rule, the patients suffer from arthritis in this case and further also from swelling of the lymph nodes. This leads to considerable restrictions in the daily life of the affected person, so that he or she is dependent on the help of other people. Everyday things can no longer be carried out without further ado. Felty’s syndrome also increases the susceptibility of patients to infections, so that they become ill more often and suffer from various infections and inflammations. This very often results in pneumonia, which leads to breathing difficulties and, if left untreated, to the death of the patient. The immune system of the affected person is generally significantly weakened and there is fatigue and exhaustion of the affected person. Various complications can also occur with pneumonia if it is diagnosed and treated late. Furthermore, Felty’s syndrome can also cause damage to the spleen, which is usually irreversible and cannot be treated. In this case, the spleen of the affected person is completely removed. Likewise, the syndrome can lead to psychological limitations or depression.
Diagnosis and course
Felty syndrome is not usually diagnosed in the primary care physician’s office, but rather at the rheumatologist’s office. It is not uncommon for patients to have gone through a long medical odyssey by this time. Only if a symptom triad is clinically detectable may the diagnosis of Felty’s syndrome be made according to the guidelines of the German Society for Rheumatology. The symptoms are symmetrical, erosive polyarthritis, neutropenia and splenomegaly. The inflammatory joint changes must therefore occur not only on one side, but on corresponding parts of the body to approximately the same extent.The term neutropenia describes the reduction of a certain fraction of white blood cells, leukocytes. Splenomegaly is a concomitant enlargement of the spleen. Overall, this immunologic event speaks to extensive and misdirected involvement of the immune system. Thus, in addition to the clinical picture, laboratory values showing a strong increase in so-called rheumatoid factors in addition to neutropenia are mandatory for diagnosis.
Complications
In most cases, women are affected by Felty syndrome; in men, the disease is largely less common and results in fewer complications and symptoms. In this case, it is mainly adults who are affected. In busy patients, Felty’s syndrome results in severe limitations of movement. Ordinary activities and sports can no longer be performed without pain or severe exertion. It also has a negative effect on the immune system. Most patients complain of a high susceptibility to infections. This can be manifested primarily by inflammation in the lungs. In the case of pneumonia, complications can arise due to late or improper treatment. However, these can also occur if the patient contracts other infections or diseases due to a weakened immune system. The lymph nodes are swollen in most affected individuals. Complications can also occur during treatment, as it is not always successful. It depends primarily on the cause of Felty’s syndrome and can result in the patient’s spleen having to be completely removed. The patient is prescribed infusions and medication, which must be taken over a longer period of time. Further symptoms depend greatly on the patient’s previous course of disease.
When should one go to the doctor?
Felty syndrome is usually treated only symptomatically. For this reason, the physician should be consulted whenever the affected person suffers from acute symptoms. Often, not all complaints can be completely limited, so that patients are dependent on prolonged treatment. A doctor should be consulted if the affected person suffers from swelling of the lymph nodes that persists over a long period of time. Different nodes may be affected by the swelling. A strong susceptibility to various infections or inflammations may also indicate Felty’s syndrome and should be investigated. Often, those affected suffer from breathing difficulties or pneumonia. Similarly, limitations in movement or general daily living may indicate the syndrome and should be investigated if they also occur over a long period of time and without a specific reason. Felty’s syndrome is usually examined and treated by a general practitioner or by an orthopedic surgeon. Furthermore, those affected may also require surgical intervention. The further course depends strongly on the manifestation of the syndrome, so that no general prediction is possible about it.
Treatment and therapy
As a special form of rheumatoid arthritis, Felty syndrome is characterized by a progressive and chronic recurrent course. Without palliative therapy, enormous suffering may manifest over time in the mostly female patients. Therapy will be based on the course of the disease, which is always dependent on the respective clinical picture, current laboratory findings or imaging to show deformities or destructions of the affected joints. Prior to therapy, a differential diagnosis should be made to exclude systemic lupus erythematosus or pseudo-Felty syndrome. This may require biopsy of inflammatory fluid from skin ulcers to confirm the diagnosis of vasculitis. Treatment, especially in the acute stage, is with high-dose corticosteroids, either orally or with the active ingredient methotrexate as infusion therapy. Due to the already reduced leukocyte count, close blood count controls are necessary during the entire therapy cycle. In addition, the injection of preparations with gold salts has proven to be a therapeutic approach. In quite a few cases, the side effects of the treatment lead to premature discontinuation of a therapy cycle. If the leukocyte count drops too much, there may also be a pronounced tendency to infection or to open and poorly healing leg ulcers.In this particularly severe form of Felty syndrome, surgical and complete removal of the spleen, splenectomy, may be indicated after critical evaluation.
Outlook and prognosis
Felty syndrome is a severe complication of rheumatoid arthritis. When this condition occurs, the outlook for recovery is usually nonexistent. Affected individuals can receive sympomatic treatment, but the physical symptoms that have already developed cannot be resolved by drug and physical therapy. The prognosis becomes worse the older the patient is. In young people, for example, there is a chance that the arthritis will improve despite Felty’s syndrome, if the prescribed medication and other accompanying measures have the desired effect. In older people, only the pain can be alleviated by the administration of appropriate preparations. In the course of the disease, other concomitant diseases such as swelling of the lymph nodes and granulocytopenia sometimes develop, which additionally worsen the prognosis. Thus, Felty’s syndrome does not offer a positive prognosis. The condition severely limits the patient’s quality of life and well-being. As a result, psychological complaints often develop, which in turn are associated with complications. Life expectancy is not reduced by Felty syndrome. However, the syndrome increases the risk of accidents and falls at home and at work.
Prevention
As a special form of rheumatoid arthritis, direct prevention of Felty syndrome is unfortunately not possible. Felty’s syndrome is a severe complication of rheumatism and is considered incurable. Rheumatics should adapt their lifestyle to their disease. Avoiding stimulant toxins and changing one’s diet cannot cure rheumatism, but they have the potential to lessen inflammatory reactions. The diet should be plant-based, and acid-forming products such as meat or sweets should be consumed only in moderation. As a preventive measure, every effort should also be made to maintain the mobility of the affected joints.
Aftercare
As a rule, the options for aftercare in Felty syndrome are severely limited, so that those affected are primarily dependent on treatment of the symptoms. This may not always result in a complete cure, so Felty syndrome may also limit or significantly reduce the life expectancy of the affected person. In most cases, however, early diagnosis has a positive effect on the course of this syndrome. Treatment often involves surgical interventions to restore the joints. No particular complications occur in this process. However, the affected person should always take it easy after these interventions and not put unnecessary strain on his body. Strenuous activities or sports should also be avoided, and stress should also be avoided. Physiotherapy measures should also be initiated after treatment of Felty’s syndrome, although many exercises from this therapy can also be performed in the patient’s own home. Since Felty’s syndrome usually requires the use of medication, care should be taken to ensure that it is taken correctly and regularly. The care and support of the affected person by outsiders also has a positive effect on the course of Felty’s syndrome and can thereby prevent psychological upsets.
What you can do yourself
In Felty syndrome, the sufferer should avoid physical overexertion or heavy strain. In everyday life, heavy strain on the joints and bones should be avoided. In addition, the muscles should be kept warm and sufficiently rested. The body should not be exposed to drafts and excess weight should be avoided. Warming baths or visits to the sauna help to alleviate the symptoms. In addition, regular sports activities are important to support the immune system. Care should be taken to practice sports that trigger a steady and gentle activation of muscles, tendons and nerves. A healthy and balanced diet helps to ward off pathogens. Physical fitness supports the organism in its daily fight against viruses or bacteria. In addition, the consumption of nicotine, alcohol or drugs should be avoided. In addition to strengthening physical power, the sick person should maintain a positive basic attitude.Optimism and confidence are important in order to be able to deal with the disease and its symptoms in everyday life in the best possible way. Relaxation techniques help to reduce stress and promote inner balance. They can be used on one’s own responsibility and according to individual wishes. In addition, the exchange with other people and joint leisure activities are important to improve the well-being.
