Still's syndrome in adults and children

For the first time this disease was described almost 120 years ago by a doctor George Still. Then, Still's syndrome was recognized as a form of rheumatoid arthritis. And only in the 70-ies of the twentieth century, Eric Bywaters presented to the medical community the data collected by him, which allowed Still's syndrome to be separated from diseases with similar symptoms.

[1], [2], [3], [4]

Epidemiology

According to the data available in the world rheumatology, the disease affects 1 person out of 100 000. Diseases are susceptible to both women and men, but children under 16 years of age are more likely to suffer.

[5], [6], [7], [8], [9], [10], [11]

Causes of the syndrome Still

A lot of studies did not give an unambiguous answer about the etiology of the Still syndrome. The onset of the development of the disease is acute, accompanied by high fever, an increase in lymph nodes and an increase in the number of leukocytes. This indicates the infectious nature of the disease. But so far, it has not been possible to identify a single causative agent. In patients, rubella virus, cytomegalovirus, parainfluenza, mycoplasma, or the Epstein-Barr virus could be found.

Physicians suggest that the disease can be due to hereditary factors. But so far this has not been precisely established. It is believed that in the pathogenesis of the disease, a certain role is played by immune deficiency. There is a theory that Still's syndrome is an autoimmune disease, but it finds confirmation only sometimes: if a blood test shows the presence of circulating immune complexes that lead to allergic vasculitis.

[12], [13]

Symptoms of the syndrome Still

Fever. The temperature rises to 39 ° C and even higher, but does not persist constantly, as with the development of other infections. Often the temperature rises rapidly one time. In rare cases, there are two temperature peaks within 24 hours. For the majority of patients between such climbs, the temperature normalizes and the well-being improves. Approximately in 1/5 of the patients the temperature does not reach normal values.

The rash in the syndrome of the Styla usually manifests itself at the peak of the heat and then disappears, then again manifests itself. It has the appearance of flat pink spots, located on the hands or feet in places where they are adjacent to the trunk, on the trunk, and sometimes - on the face. Approximately one-third of the cases of the syndrome of the Steele rash rises on the skin and appears where the skin was injured or squashed. The mild color of the rash, its episodic disappearance and the absence of itching, dryness and other manifestations leave the rash unnoticed for the patients.

Sometimes a physician has to examine a patient after a warm shower or use another exposure to heat to detect a rash. But with Still's syndrome there are also atypical manifestations: petechiae, erythema nodosum, hair loss.

Pain in the joints. Joint pain, like muscle pain, is referred to the symptoms at the onset of the development of the syndrome, which are caused by an increase in temperature. First, arthritis can manifest only in one joint. After a while, it affects other joints: not only in the limbs, but also the jaws. The most characteristic of the syndrome is the arthritis of the joints between the phalanges. This manifestation makes it possible to differentiate the disease from rheumatoid arthritis, rheumatic fever, lupus erythematosus, in which these joints do not suffer in children.

The defeat of the organs of the lymphatic system. This is a simultaneous increase in the liver and spleen, as well as inflammation of the lymph nodes. Lymph nodes are inflamed in 2/3 of the patients. For ½ cases, the cervical lymph nodes increase. In this case, the lymph nodes remain mobile and moderately dense. Strong lymph node consolidation, an increase in only one lymph node and adhesion to neighboring tissues is an occasion to seek advice from an oncologist. Sometimes inflammation can be accompanied by necrotic phenomena.

Two thirds of patients with Still's syndrome complain of a sore throat. Pain makes itself felt at the beginning of the development of the disease: burning in the throat persists constantly.

Disorders of the heart and lungs. Often expressed in the form of serositis, when the serous membrane of these organs becomes inflamed. In 1/5 of cases, pneumonitis is detected, which is not infectious, and proceeds as bilateral pneumonia: with coughing, fever and dyspnea, and taking antibiotics does not give results. Less often with Still's syndrome, there is a cardiac tamponade, inflammation of the heart muscle, signs of vegetation of microorganisms on the heart valve, acute respiratory failure.

Symptoms of the Still syndrome in children do not differ from the symptoms in adults, but they may not manifest so distinctly, because of which the diagnosis is established late and the correct treatment is prescribed. Polyarthritis can lead a child to disability. Launched cases of the Still syndrome in childhood can cause disproportionate growth of the arms and legs, which can only be corrected by surgery.

Diagnostics of the syndrome Still

The pathogenesis of the Still's syndrome does not have any particular signs that allowed to accurately detect the disease. Patients often diagnose sepsis, although blood tests do not show the presence of bacteria. It is noted that in some cases, the doctor initially diagnosed a fever of unknown origin.

Only after conducting several courses of antibiotics and doing additional research, doctors diagnose Still's syndrome in adults. Considered as manifestations in the form of high temperature, swelling of the joints, enlarged lymph nodes and pain in the throat, and data of instrumental diagnostics: cardiogram, MRI and ultrasound. A typical non-erosive narrowing of the wrist-metacarpal and intercostal joints for Styll syndrome can be detected using radiography.

Blood tests for the disease show a reduced level of erythrocytes and a very high white blood cell count. In patients, the level of C-reactive protein and ferritin protein is too high, and the tests for antinuclear antibodies and rheumatoid factor give a negative result.

For diagnosis, several groups of diagnostic features are suggested, but in practice they are guided by the criteria of Kasha. The peculiarity of the disease is that almost always at the beginning of the syndrome there is no complete clinical picture. A common first sign is fever, and other symptoms progress weeks or even months. In a patient with a set of symptoms, where there is fever, pain in the muscles and joints, a rash, elevated leukocyte counts - it is unlikely that any other disease develops, other than the Still's syndrome of adults. Therefore, this diagnosis is the first in the list of diseases of differential diagnosis. Almost all other diseases can be excluded by clinical data and simple diagnostic tests.

[14], [15], [16], [17]

Treatment of the syndrome Still

Therapy during an exacerbation

Usually non-steroidal anti-inflammatory drugs (NSAIDs), including acetylsalicylic acid, are prescribed. Such treatment does not give fast results, but in almost all cases it is possible to talk about a good prognosis.

• Acetylsalicylic acid is prescribed in a dosage of 60-80 mg / kg / day. It is taken after meals 3-4 times a day. The duration of the course is from one to three months and depends on how well the patient tolerates.
• Indomethacin is prescribed in a dose of 2-3 mg / kg / day.
• Diclofenac is taken at 2-3 mg / kg / day, dividing into 2 doses.
• Ibuprofen is administered at a dosage of 200 to 1000 mg / day. Dosage depends on the age of the patient and is taken from the calculation of 40 mg / kg / day. The drug is divided into 3 receptions.
• Naproxen is prescribed at 250-750 mg / day, depending on the age. Treatment with the drug is not recommended for patients younger than 10 years. Duration of treatment - from several months to several years.

The main problem that accompanies taking these drugs is structural and functional disorders of the liver. These consequences may be manifestations of the disease itself, and the results of liver tests can come to normal, despite prolonged treatment with nonsteroidal anti-inflammatory drugs. Patients undergoing treatment are monitored liver function both in the hospital and after the completion of the course of treatment. These drugs increase the likelihood of thrombohemorrhagic complications.

If treatment with anti-inflammatory drugs was not successful, if the patient develops systemic intravascular coagulopathy against the background of the Still's syndrome, or hepatic tests show a disruption of the liver during therapy with nonsteroidal anti-inflammatory drugs, hormonal treatment may be required. In the first place, usually prescribed prednisone intake of 0.5-1 mg per kilogram of body weight daily. However, with a reduction in dosage, the disease can manifest itself with renewed vigor, moreover, prolonged treatment does not prevent further destruction of the joints.

If Still's syndrome develops in a form that can be life threatening, methylprednisolone or betamethasone therapy is administered intra-articularly. The dosage of drugs depends on the size of the joint. In the joint, the drug is administered up to 5 times with an interval of 5 days, then you can repeat the course.

How to treat the chronic form of the Still's syndrome?

Often, the cause of the chronic course of the syndrome is arthritis.

Methotrexate. To control arthritis and chronic systemic process, it is recommended to take methotrexate weekly in small doses. The recommended initial dose is 7.5 mg. It can be divided into 3 receptions with a break at 12 o'clock or taken once. Once the clinical effect is achieved, the dosage is reduced to the lowest effective dose.

• The drug also has a negative effect on liver function, but for 70% of patients this treatment is effective. The drug may increase the susceptibility of the skin to ultraviolet radiation. During therapy with methotrexate, prolonged exposure to sunlight or ultraviolet radiation should not be allowed. To do this, it is necessary to wear closed clothing, glasses from the sun, use sunscreen cosmetics.
• Hydroxychloroquine. With moderate manifestations of the chronic form of the syndrome of the Still syndrome (eg, loss of strength, fever, rashes, serositis) hydroxychloroquine may be effective. The drug can be taken concomitantly with methotrexate. The daily dose of the drug - 400-600 mg, divided into several receptions, then the dosage can be reduced. Care should be taken when prescribing the drug to patients with liver and kidney dysfunction, gastrointestinal disorders, neurological diseases, psoriasis, sensitivity to quinine. If the patient takes medication, whose adverse reactions can affect the skin or vision, the appointment of hydroxychloroquine should also be cautious.

There is evidence of increased toxicity of sulfasalazine, which limits its use.

If treatment with these drugs has not produced a result, drugs that depress immunity are prescribed:

• Azathioprine - 1.5-2 mg / kg in 2-4 admission. The duration of the course is set separately in each case. The drug is contraindicated in the violation of hematopoiesis, violations of the liver. Taking the drug in any case can not be canceled arbitrarily, as the disease can worsen.
• Cyclophosphamide. To obtain an immunosuppressive effect, the drug is taken in a dosage of 1-1.5 mg per kg of body weight daily. During therapy with cyclophosphamide, it is recommended to drink plenty of fluids. The patient must strictly adhere to the instructions for taking the drug, the therapy is always under the supervision of the doctor. Prior to the start of the course of treatment, it is necessary to monitor blood levels, liver and kidney function. During treatment with immunosuppressants, you should carefully do a manicure, avoid skin trauma, gently brush your teeth, do not contact sick people, do not get vaccinated.

As a result of taking cyclophosphamide, a false positive reaction to candidiasis, tuberculin test, and parotitis may occur.

• Ciclosporin A. Take 3 mg per kilogram of weight for the first 45 days. Then the dosage can be reduced to a minimum, but at the same time the therapeutic effect remains. The course of treatment is up to 3 months. The drug inhibits the production of antibodies, slows the response of T-helpers, selectively and reversibly changes the function of lymphocytes. The drug does not affect the processes of hematopoiesis and the number and functional activity of neutrophils of the immune system.

For therapy, the administration of an immunoglobulin - alone or in combination of mycophenolate with mycophenolate - is also used. However, this treatment is not always successful.

Not so long ago, the drugs blocking the enzyme monoamine oxidase factor of tumor necrosis factor (TNF-alpha) began to be used in the therapy of the Still syndrome. These medications of this group (infliximab, adalimumab, etanercept) help to relieve inflammation. These are the newest developments in therapy, the drugs are very expensive, they do not always give the expected result, but sometimes they can ideally approach patients.

Also, when Steele's disease is used, local therapy is used when the affected joints are treated: drugs are injected into the joint, mainly glucocorticoids, for a time they immobilize the joint using a lantetum, use a wide range of physiotherapy, physiotherapy, massage. If there are contractures, apply skeletal traction, as well as mechanotherapy on specialized devices.

Some patients try to support their body with vitamins, homeopathic preparations, but the effectiveness of these drugs in the fight against the syndrome of the Styla is not proven and they can be used only as maintenance therapy.

Operative treatment

Conservative methods of treatment continue to develop, but also often the manifestations of the disease are surgically treated. Surgery is performed at an early stage with the purpose of resection of the synovial membrane of the joint. This stops the inflammatory process, while keeping the cartilage from imminent damage, which allows you to save the joint and restore its work.

Elimination of granulation tissue reduces the autoimmune response of the body, positively affects the well-being, provides a stable remission.

Synovectomy is used if a complex of antirheumatic conservative treatment for a long time - six months or longer - did not bring positive results.

Alternative Treatment for Still's Syndrome

• Salt. If the joints "break", alternative medicine recommends rubbing them with salt, which is previously dissolved in honey or vodka.
• Clay. Of some varieties of clay, which occur in certain places, make appliqués. To do this, you need a greasy, plastic clay. It is diluted with water, stirring to a creamy consistency. For applications, the clay must be warm-40-48 oC. Application is done up to 5 cm in thickness. Apply for 15-30 minutes. The course of treatment 12-20 sessions, which are held every other day.
• Paraffin. First, it is melted by means of a water bath, and then allowed to cool to the required temperature. The most acceptable method of home treatment is when the melted paraffin is poured into a container to make an application 1-2 cm thick. The congealing but still plastic cake (temperature of 50-55 ° C) is taken out of the container and the application is made, which is wrapped with a warm cloth. Duration of the session - half an hour-hour, paraffin therapy is recommended every other day. Duration of treatment - 10-30 sessions. During each session, apply the application for 2-3 joints at the same time - no more, then alternate.
• Honey. Mix honey, aloe juice and vodka in a 2: 1: 3 ratio. Use as a compress, which helps to relieve inflammation in the joints.

[18], [19], [20], [21], [22], [23], [24], [25]

Herbal Treatment

• Reception of pine baths. Branches, needles and cones of pine are flooded with water, brought to a boil and boil for half an hour on low heat. Then, wrapping the container in a warm cloth, insist 10-12 hours. Coniferous broth should be brown. Immediately you can make a medical inhalation, adding to the bath 20 drops of essential oil of pine.
• Physalis vulgaris removes inflammation and pain. Infusion is prepared from dried fruits: take 2 tablespoons per half liter of boiling water and boil for 5 minutes at minimum heat. Take 100 ml 3-4 times a day for 15-20 minutes before meals.
• Cucumber has anti-inflammatory effect. Two tablespoons of herbs pour 2 glasses of water and insist 4-5 hours. Take a tablespoon 5-6 times a day.
• Cherry. Herbalists to relieve pain in the joints recommend breaking the bones of cherries, removing seeds, drying, grinding and making compresses.
• Bay leaf. 1/2 pack of dry bay leaf is poured with 300 grams of cooled boiled water. Then bring to a boil and cook at minimum heat for 5 minutes. Slightly cool and drink the whole broth immediately. The course of treatment is 3 days.

Forecast

The result of the syndrome may be full recovery, the development of a recurrent or chronic form. Recover about 30% of patients, often within a few months from the time of the disease. Approximately 20% of patients experience prolonged remission - up to 1 year. In 30% of patients, symptoms recede, but one or more exacerbations occur later. The recurrent form of the Still syndrome in 70% of patients means a single exacerbation, which can occur after 10 months or even 10 years. When a relapse occurs, it is impossible to predict, but often it is easier and faster than the first episode. Some patients may experience a cyclical recurrence of the disease with recurring attacks. The most severe is the chronic form that develops in the remaining patients. It proceeds with severe polyarthritis. According to observations, the manifestation of arthritis symptoms at an early age is an unfavorable prognostic sign.

Among adult patients with the disease, the survival rate for five years is 90-95%. Patients may die due to the development of secondary infection: violations of blood coagulation, cardiac, liver, inflammatory processes in the lungs, tuberculosis.

Still's syndrome is a rare, but serious, disease that can lead to disability. It often affects young people, which increases its destructive character. A competent and attentive doctor can play a decisive role. Remember that the symptoms can recede a few years after the onset of the disease and that most of the patients live a full life even years after the diagnosis was made.

[26], [27], [28], [29], [30], [31],