Juvenile rheumatoid arthritis in children: Still's syndrome - features of the clinical course, modern approaches to therapy

What is Still's disease?

Still's disease is considered a rare and serious disease. Arthritis is characterized by joint pain,

but in this case, the patient has a sore throat, body temperature rises and an orange-pink rash appears on the skin. To recognize the disease, doctors conduct laboratory tests and check the lymphoreticular and cardiopulmonary systems. Particular attention is paid to the joints. Therapy includes taking nonsteroidal drugs that relieve inflammation, glucocorticosteroids, and cytostatics. Timely assistance eliminates the development of complications. But first, patients must understand that they are dealing with Still's disease.

How to recognize the disease?

If an adult gets sick with a rare type of arthritis, he notices the following changes in the body:

1. Sore throat. The cervical lymph nodes become swollen and inflamed, touching them becomes painful.
2. Body temperature rises. The indicator reaches its maximum in the morning and evening.
3. Skin rashes. An orange-pink rash appears on the arms, legs and body. This is a consequence of high body temperature.
4. Joints hurt, swell, become inflamed. A person feels stiffness in the elbows, knees, and shoulders.
5. Muscle discomfort appears. This is caused by high temperature.
6. Lymphadenopathy and hepatosplenomegaly develop. Illnesses appear in 70% of patients with Still's disease.

What complications should you be wary of?

The situation is complicated by the fact that over time, inflammation of the joints and organs becomes chronic. Other complications include:

1. Joint syndrome. The knee and wrist joints are destroyed.
2. Inflammation of the heart muscle. Myocarditis and pericarditis develop.
3. Accumulation of fluid in the lungs. Taking deep breaths becomes difficult.

Why does the disease appear?

The etiology of the disease is not fully understood. Many doctors believe that Still's disease is infectious in nature. Associations arise with parainfluenza, rubella virus, and mycoplasma. Pathogenic microbes are one of the causes of the development of the disease. The other is hereditary predisposition. Still's disease is also classified as an autoimmune disease.

How to diagnose a rare type of arthritis?

Examination of the patient is the task of the rheumatologist. The doctor monitors the patient’s condition and rules out diseases with similar symptoms. Blood donation is required. Biochemical and clinical analysis is carried out in laboratory conditions. The deformed joint can be seen on x-ray. Sometimes a lymph node biopsy is performed. If heart problems occur, the patient should consult a pulmonologist and cardiologist. In addition to X-rays of the lungs, an ECG and ultrasound will be prescribed.

How to cure Still's disease?

Medicines will help cope with the disease. To stop pathological changes, it is necessary to take non-steroidal anti-inflammatory drugs. In a quarter of cases, this is enough to stop further exacerbation. If the disease affects the cardiopulmonary system, glucocorticoids are prescribed. A rare type of arthritis is considered an autoimmune disease, so the doctor prescribes immunostimulants. Other medications suitable for treatment:

1. Interferon;
2. Sulfanilamide;
3. Gold preparations;
4. Cytostatics;
5. Cyclosporine A;
6. Means for systemic enzyme therapy;
7. Aminoquinoline drugs.

In case of severe liver damage, hepatoprotectors are prescribed. In advanced cases (with unbearable pain), patients are injected with glucocorticoids into the joint cavity. Protease inhibitors are sometimes used. Treatment is also carried out at the local level: the doctor prescribes gels and ointments against inflammation, and prescribes physical therapy. An additional treatment method is plasmapheresis.

What are the chances of recovery?

A third of patients cope with the disease within nine months, sometimes earlier.
The remaining patients suffer from periodic exacerbations and risk developing a chronic form of Still's disease. Death is possible only in the presence of secondary infections. Author: K.M.N., Academician of the Russian Academy of Medical Sciences M.A. Bobyr

School of Rheumatology

“When we talk about rare diseases, issues of differential diagnosis always come to the fore, because it is often extremely difficult to give the patient the correct diagnosis during the initial examination,” notes Professor, Director of the Federal State Budgetary Institution NIIR. V. A. Nasonova, Doctor of Medical Sciences Alexander Lila.

The expert explains that such care can only be provided to patients in an institution with a multidisciplinary team, where there are all the capabilities to carry out diagnostics and provide high-tech medical care within the same walls.

“If we talk about the Russian Federation as a whole, then specialists from the regions are often in dire need of advice to determine the algorithm for managing patients with Still’s disease. It will now be easier to obtain it thanks to the opening of a reference center for autoinflammatory diseases on the website of our institution,” says Lila. “Now a doctor of any specialty can receive correspondence consultation from federal experts if EFS is suspected in patients over 18 years of age.”

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Care

Still's disease in adults is treated with anti-inflammatory drugs. Steroids such as prednisone are used to treat severe Still's symptoms. Other commonly used medications include: hydroxychloroquine, penicillamine, azathioprine, methotrexate, etanercept, Anakinra, tocilizumab cyclophosphamide, adalimumab, rituximab, and infliximab.[12]

New drugs target interleukin-1 (IL-1), especially IL-1β.[13] A randomized multicenter trial found better results in a group of 12 patients treated with anakinra than in a group of 10 patients treated with other disease-modifying antirheumatic drugs.[14] In June 2021, the FDA approved Ilaris (canakinumab) for the treatment of AOSD, the first FDA-approved treatment for AOSD.[15] Canakinumab is another anti-IL1 drug that selectively binds IL-1β and rilonacept, which blocks both IL-1A and IL-1β.[16] The anti-IL6 monoclonal antibody tocilizumab is another treatment option that is as effective as anakinra.[17]

The condition "juvenile-onset Still's disease" is now commonly grouped under juvenile rheumatoid arthritis. However, there is some evidence that the two conditions are closely related.[18][19]