Still's syndrome in adults and children

The disease was first described almost 120 years ago by physician George Still. At that time, Still's syndrome was recognized as a form of rheumatoid arthritis. It was only in the 1970s that Eric Bywaters presented the medical community with the data he had collected, which allowed Still's syndrome to be separated from diseases with similar symptoms.

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Epidemiology

According to data available to global rheumatology, the disease affects 1 in 100,000 people. Both women and men are susceptible to the disease, but children under 16 are more often affected.

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Causes Still's syndrome

Numerous studies have not provided a clear answer about the etiology of Still's syndrome. The onset of the disease is acute, accompanied by high temperature, enlarged lymph nodes and an increase in the number of leukocytes. This indicates an infectious nature of the disease. But so far it has not been possible to identify a single pathogen. Patients could have rubella virus, cytomegalovirus, parainfluenza, mycoplasma or Epstein-Barr virus.

Doctors assume that the disease may be caused by hereditary factors. But this has not yet been established with certainty. It is believed that immune deficiency plays a certain role in the pathogenesis of the disease. There is a version that Still's syndrome is an autoimmune disease, but it is confirmed only sometimes: if a blood test shows the presence of circulating immune complexes that lead to allergic vasculitis.

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Symptoms Still's syndrome

Fever. The temperature rises to 39°C or even higher, but does not remain constant, as with other infections. Often the temperature rises rapidly once. In rare cases, there are two temperature peaks within 24 hours. In most patients, the temperature normalizes between such rises and the patient feels better. In approximately 1/5 of patients, the temperature does not reach normal values.

The rash in Still's syndrome usually appears at the peak of the fever and then disappears and reappears. It looks like flat pink spots located on the arms or legs where they adjoin the torso, on the torso itself, and sometimes on the face. In about a third of cases of Still's syndrome, the rash is raised on the skin and appears where the skin has been injured or compressed. The dull color of the rash, its episodic disappearance, and the absence of itching, dryness, and other manifestations leave the rash unnoticed by patients.

Sometimes a physician has to examine a patient after a warm shower or use other heat to detect rashes. But Still's syndrome can also have atypical manifestations: petechiae, erythema nodosum, hair loss.

Joint pain. Joint pain, like muscle pain, at the beginning of the syndrome development is attributed to symptoms caused by the rise in temperature. At first, arthritis may manifest itself in only one joint. After a while, it affects other joints: not only in the limbs, but also the jaw. The most characteristic of Still's syndrome is arthritis of the joints of the hand 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.

Damage to the lymphatic system organs. This is a simultaneous enlargement of the liver and spleen, as well as inflammation of the lymph nodes. The lymph nodes become inflamed in 2/3 of patients. Enlargement of the cervical lymph nodes is typical for ½ of cases. In this case, the lymph nodes remain mobile and moderately dense. Severe compaction of the lymph node, enlargement of only one lymph node and adhesion to adjacent tissues are a reason to consult an oncologist. Sometimes inflammation can be accompanied by necrotic phenomena.

2/3 of patients with Still's syndrome complain of a sore throat. The pain makes itself known at the beginning of the disease: the burning sensation in the throat remains constant.

Heart and lung dysfunction. Often manifested as serositis, when the serous membrane of these organs becomes inflamed. In 1/5 of cases, pneumonitis is detected, which is not infectious in nature and proceeds as bilateral pneumonia: with cough, fever and shortness of breath, and antibiotics do not give results. Less common with Still's syndrome are cardiac tamponade, inflammation of the heart muscle, signs of microbial vegetation on the heart valve, and acute respiratory failure.

Symptoms of Still's syndrome in children are no different from those in adults, but they may not be as obvious, which is why diagnosis and proper treatment are delayed. Polyarthritis can lead to disability in a child. Advanced cases of Still's syndrome in childhood can cause disproportionate growth of the arms and legs, which can only be corrected by surgery.

Diagnostics Still's syndrome

The pathogenesis of Still's syndrome does not have any specific signs that would allow the disease to be accurately identified. Patients are often diagnosed with sepsis, although blood tests do not show the presence of bacteria. It is noted that in some cases the doctor initially diagnosed fever of unknown genesis.

Only after several courses of antibiotics and additional examinations, doctors diagnose Still's syndrome in adults. Both manifestations in the form of high temperature, joint swelling, enlarged lymph nodes and sore throat are taken into account, as well as instrumental diagnostic data: cardiogram, MRI and ultrasound. Nonerosive narrowing of the carpometacarpal and intercarpal joints, typical of Still's syndrome, can be detected using radiography.

Blood tests show low red blood cell counts and very high white blood cell counts. Patients have elevated levels of C-reactive protein and ferritin, and tests for antinuclear antibodies and rheumatoid factor are negative.

Several groups of diagnostic signs have been proposed for diagnosis, but in practice, the Kasch criteria are used. The peculiarity of the disease is that almost always at the beginning of the development of the syndrome there is no complete clinical picture. A common first sign is fever, and other symptoms progress over weeks or even months. In a patient with a set of symptoms, including fever, muscle and joint pain, rash, and elevated white blood cell count, it is unlikely that any other disease is developing, except for adult Still's syndrome. Therefore, this diagnosis is first in the list of diseases of differential diagnosis. Almost all other diseases can be excluded based on clinical data and simple diagnostic tests.

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Treatment Still's syndrome

Therapy during exacerbation

Nonsteroidal anti-inflammatory drugs (NSAIDs), including acetylsalicylic acid, are usually prescribed. This treatment does not give quick results, but in almost all cases we can 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 it.
• Indomethacin is prescribed at a dose of 2-3 mg/kg/day.
• Diclofenac is taken at 2-3 mg/kg/day, divided into 2 doses.
• Ibuprofen is prescribed in a dosage of 200 to 1000 mg/day. The dosage depends on the patient's age and is calculated at 40 mg/kg/day. The drug is taken in 3 doses.
• Naproxen is prescribed at 250-750 mg/day depending on age. Treatment with the drug is not recommended for patients under 10 years of age. The duration of treatment is from several months to several years.

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

If treatment with anti-inflammatory drugs has not been successful, if the patient develops systemic intravascular coagulopathy against the background of Still's syndrome, or if liver function tests show liver dysfunction during therapy with nonsteroidal anti-inflammatory drugs, treatment with hormonal drugs may be required. First of all, prednisone is usually prescribed at 0.5 - 1 mg per kilogram of body weight daily. However, if the dosage is reduced, the disease may manifest itself with renewed vigor, and long-term treatment does not prevent further destruction of joints.

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

How to treat chronic Still's syndrome?

Often the cause of chronic Still's 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 of the drug is 7.5 mg. It can be divided into 3 doses with a break of 12 hours or taken once. As soon as the clinical effect is achieved, the dosage is reduced to the minimum effective dose.

• The drug also has a negative effect on liver function, but for 70% of patients, this treatment is effective. The drug can increase the skin's sensitivity to ultraviolet radiation. During methotrexate therapy, prolonged exposure to sunlight or ultraviolet radiation should be avoided. To do this, you must wear closed clothing, sunglasses, and use sunscreen cosmetics.
• Hydroxychloroquine. In moderate cases of chronic Still's syndrome (e.g. fatigue, fever, rash, serositis), treatment with hydroxychloroquine may be effective. The drug can be taken simultaneously with methotrexate. The daily dose of the drug is 400-600 mg, divided into several doses, then the dosage can be reduced. Caution should be exercised when prescribing the drug to patients with liver and kidney dysfunction, gastrointestinal disorders, neurological diseases, psoriasis, sensitivity to quinine. If the patient is taking medications whose side effects may affect the skin or vision, caution should also be exercised when prescribing hydroxychloroquine.

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

If treatment with these drugs does not bring results, drugs that suppress the immune system are prescribed:

• Azathioprine – 1.5-2 mg/kg in 2-4 doses. The duration of the course is determined separately in each case. The drug is contraindicated in case of hematopoietic dysfunction, liver dysfunction. In no case should the drug be stopped arbitrarily, as the disease may worsen.
• Cyclophosphamide. To obtain an immunosuppressive effect, the drug is taken in a dosage of 1-1.5 mg per kg of weight daily. During cyclophosphamide therapy, it is recommended to drink plenty of fluids. The patient must strictly follow the instructions for taking the drug; therapy must be carried out under the supervision of a doctor. Before starting the course of treatment, it is necessary to monitor blood counts, liver and kidney function. During treatment with immunosuppressants, you should carefully do a manicure, avoid skin injuries, brush your teeth carefully, avoid contact with sick people, and do not get vaccinated.

Taking cyclophosphamide may result in a false-positive reaction to candidiasis, tuberculin test, and mumps.

• Cyclosporine A. Take 3 mg per kilogram of weight for the first 45 days. Then the dosage can be reduced to a minimum, but so that the therapeutic effect is preserved. The course of treatment is up to 3 months. The drug inhibits the mechanism of antibody production, slows down the reactions 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.

Immunoglobulin is also used for therapy, either alone or in combination with mycophenolate mofetil. However, this treatment is not always successful.

Not long ago, drugs that block the enzyme tumor necrosis factor monoamine oxidase (TNF-alpha) began to be used in the treatment of Still's syndrome. These drugs of this group (infliximab, adalimumab, etanercept) help relieve inflammation. These are the latest developments in therapy, the drugs are very expensive, do not always give the expected result, but sometimes they can be ideal for patients.

Also, for Still's disease, local therapy is used when treating the affected joints: drugs are injected into the joint, mainly glucocorticoids, the joint is immobilized for a while using a splint, a wide range of physiotherapeutic treatment methods, exercise therapy, and massage are used. If contractures are present, skeletal traction is used, as well as mechanotherapy on specialized devices.

Some patients try to support their bodies with vitamins and homeopathic medicines, but the effectiveness of these drugs in the fight against Still's syndrome has not been proven and they can only be used as supportive therapy.

Surgical treatment

Conservative treatment methods continue to develop, but manifestations of Still's disease are also often treated surgically. Surgical intervention is performed at an early stage to resect the synovial membrane of the joint. This stops the inflammatory process, while preserving the cartilage from inevitable damage, which allows you to save the joint and restore its function.

Elimination of granulation tissue reduces the body's autoimmune response, has a positive effect on well-being, and ensures stable remission.

Synovectomy is used if a complex of antirheumatic conservative treatments over a long period of time – six months or longer – has not produced positive results.

Folk treatment of Still's syndrome

• Salt. If your joints ache, traditional medicine recommends rubbing them with salt, which has been previously dissolved in honey or vodka.
• Clay. Some types of clay that are found in certain places are used for applications. This requires fatty, plastic clay. It is diluted with water, stirring until it reaches a creamy consistency. For applications, the clay should be warm - 40-48 oC. The application is made up to 5 cm thick. It is applied for 15-30 minutes. The course of treatment is 12-20 sessions, which are held every other day.
• Paraffin. First, it is melted using a water bath, and then allowed to cool to the required temperature. The most acceptable method of home treatment is when melted paraffin is poured into a container to make an application 1-2 cm thick. The solidifying, but still plastic cake (temperature 50-55 oC) is taken out of the container and an application is made, which is wrapped in a warm cloth. The duration of the session is half an hour to an hour, it is recommended to conduct paraffin therapy every other day. The duration of treatment is 10-30 sessions. During each session, the application is applied to 2-3 joints at the same time - no more, then alternate.
• Honey. Mix honey, aloe juice and vodka in a ratio of 2:1:3. Use as a compress that helps relieve inflammation in the joints.

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Herbal treatment

• Taking pine baths. Pine branches, needles and cones are poured with water, brought to a boil and boiled for half an hour on low heat. Then, wrapping the container in a warm cloth, leave for 10-12 hours. The coniferous decoction should be brown. You can immediately do a therapeutic inhalation by adding 20 drops of pine essential oil to the bath.
• Physalis vulgaris relieves inflammation and pain. An infusion is prepared from dried fruits: take 2 tablespoons per half a liter of boiling water and boil for 5 minutes at minimum heat. Take 100 ml 3-4 times a day 15-20 minutes before meals.
• Cucumber grass has an anti-inflammatory effect. Two tablespoons of the herb are poured with 2 glasses of water and infused for 4-5 hours. Take a tablespoon 5-6 times a day.
• Cherry. Herbalists recommend breaking cherry pits, removing the seeds, drying, grinding and making compresses to relieve joint pain.
• Bay leaf. Pour 1/2 pack of dry bay leaf with 300 grams of cooled boiled water. Then bring to a boil and simmer at minimum heat for 5 minutes. Cool slightly and drink the entire decoction at once. The course of treatment is 3 days.

Forecast

The outcome of Still's syndrome may be complete recovery, development of a recurrent or chronic form. About 30% of patients recover, often within a few months from the onset of the disease. About 20% of patients experience long-term remission - up to 1 year. In 30% of patients, the symptoms subside, but then one or more exacerbations occur. The recurrent form of Still's syndrome in 70% of patients means a single exacerbation, which may occur after 10 months or even after 10 years. It is impossible to predict when a relapse will occur, but it is often easier and faster than the first episode. Some patients may experience a cyclical recurrent course of the disease with repeated attacks. The most severe is the chronic form, which develops in other patients. It occurs with severe polyarthritis. According to observations, the manifestation of arthritis symptoms at an early age is an unfavorable prognostic sign.

Among adult patients with Still's disease, the five-year survival rate is 90-95%. Patients may die due to the development of a secondary infection: blood coagulation disorders, heart and liver dysfunction, inflammatory processes in the lungs, tuberculosis.

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

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