Juvenile rheumatoid arthritis is a rheumatic disease that begins before the age of 16 years. Arthritis, fever, skin rashes, lymphadenopathy, splenomegaly and iridocyclitis are typical symptoms. Diagnosis is made by analyzing clinical manifestations. Treatment uses NSAIDs, often antirheumatic drugs that modify the course of the disease.
Juvenile rheumatoid arthritis is uncommon. The cause is unknown, but genetic predisposition and an autoimmune response are believed to play an important role. The clinical picture of juvenile rheumatoid arthritis may be similar to that of rheumatoid arthritis in adults.
Patients with juvenile rheumatoid arthritis experience joint stiffness, swelling, pain, and the development of intra-articular effusion. Juvenile rheumatoid arthritis can affect the body's growth and development. Due to the early closure of the growth zones of the lower jaw, micrognathia (underdevelopment of the lower jaw) is noted. Iridocyclitis develops, which leads to infections of the conjunctiva, pain and photophobia, but can also be asymptomatic; the consequence may be the development of scarring of the cornea, glaucoma and keratopathy. There are three variants of the course of juvenile rheumatoid arthritis.
Juvenile rheumatoid arthritis should be suspected in children who present with arthritis, iridocyclitis, generalized lymphadenopathy, splenomegaly, or unexplained fever for several days. The diagnosis is established by analyzing the clinical manifestations. Patients suspected of having juvenile rheumatoid arthritis should be examined for the presence of rheumatoid factor and antinuclear antibodies with determination of ESR, since these tests are useful in diagnosing juvenile rheumatoid arthritis and determining the course of the disease. In Still's disease, rheumatoid factor and AHA are not detected. In oligoarthritis, AHAs are present in almost 75% of patients. With polyarticular lesions, rheumatoid factor is usually negative, but in some patients (mostly adolescent girls) it can be detected.
To diagnose iridocyclitis, a slit-lamp examination should be performed even in the absence of ocular manifestations. For oligoarthritis, an ophthalmological examination should be carried out once every 3-4 months, for polyarthritis - once every 6 months.
With adequate treatment, 50-75% of patients achieve complete remission. Patients suffering from polyarticular disease in whom rheumatoid factor is detected have a less favorable prognosis.
As with the treatment of rheumatoid arthritis, disease-modifying antirheumatic drugs are used in adults, in particular biological drugs, which can significantly change the outcome.
Symptomatic therapy is carried out by prescribing NSAIDs. Naproxen (orally 5-10 mg/kg 2 times a day), ibuprofen (orally 5-10 mg/kg 4 times a day), indomethacin (orally 0.5-1.0 mg/kg 4 times a day) are used. Salicylates are rarely used due to the risk of developing Reye's syndrome.
Systemic glucocorticoids are not usually used except in cases of systemic disease. If their use is necessary, the lowest possible dose should be prescribed (for example, prednisolone orally 0.0125-0.5 mg/kg 4 times a day; this dose can be divided into 1-2 doses). The most serious complications of long-term glucocorticoid therapy in children are growth retardation, development of osteoporosis and osteonecrosis. Intra-articular administration of glucocorticoids is allowed, the dose of which in this case should be adjusted based on the size of the joint.
For polyarticular disease, methotrexate and hydroxychloroquine can be used. Monitor for side effects in the same way as in adults. The degree of bone marrow suppression and the development of toxic hepatitis is monitored by assessing the amount of cellular composition of peripheral blood, AST, ALT activity and albumin concentration. During treatment with hydroxychloroquine, monitoring of changes in visual fields is necessary. Rarely, especially if the development of spondyloarthropathy is suspected, sulfasalazine is used. Intramuscular administration of gold and penicillamine drugs is rarely used.
Etanercept, used as in adults, blocks tumor necrosis factor and is often effective; administered subcutaneously at a dose of 0.4 mg/kg (with an increase to a maximum of 25 mg) 2 times a week. Physical therapy, removable splints and other measures help avoid flexion contractures. Various assistive devices help improve function and minimize stress on affected joints. For iridocyclitis, eye drops containing glucocorticoids and mydriatics are used.
Juvenile rheumatoid arthritis (JRA) is arthritis of unknown cause, lasting more than 6 weeks, developing in children under the age of 16 years when other joint pathology is excluded.
Juvenile rheumatoid arthritis is an autoimmune disease, which means the immune system mistakenly perceives its own body tissues as foreign and tries to destroy them. However, experts still do not know why such a malfunction of the immune system occurs. It is assumed that infectious diseases and preventive vaccinations, especially against measles, mumps or rubella, can trigger the disease. Moreover, after vaccination, girls are more likely to develop rheumatoid arthritis. However, infection is not the only cause of juvenile rheumatoid arthritis. It can also develop after joint injuries, hypothermia, or overheating in the sun.
In juvenile rheumatoid arthritis, non-purulent inflammation of the synovial membranes occurs. In this case, joint fluid is released in increased quantities and accumulates in the joint cavity. The synovial membrane thickens, adheres to the articular cartilage and after some time fuses with it. Cartilage erosion occurs. And after some time the cartilage is destroyed. Moreover, the time during which these changes occur varies for all patients.
Based on the number of affected joints, they are classified into:
Oligoarthritis can be of two types – first and second. 80% of children suffering from type 1 oligoarthritis are girls. The disease begins at a young age, usually affecting the ankle, knee and elbow joints. The disease is accompanied by chronic iridocyclitis. Rheumatoid factor (autoantibodies to immunoglobulin G) is absent. In 10% of cases, children remain with vision problems for the rest of their lives, and in 20% of cases – with joints.
Oligoarthritis of the second type mainly affects boys. It develops later than type 1 oligoarthritis. In this case, large joints are affected, most often the hip. The disease is often accompanied by sacroiliitis (inflammation of the sacroiliac joint), in 10-20% of cases it is accompanied by acute iridocyclitis. Rheumatoid factor is absent. Often, children who have had type 2 oligoarthritis still have spondyloarthropathy (a disease of the joints and the places where tendons attach to the bones).
Polyarthritis is divided into rheumatoid factor positive and negative. It is mostly girls who suffer from both. Polyarthritis negative for rheumatoid factor can develop in children at any age, and it affects any joints. Occasionally, the disease is accompanied by iridocyclitis (inflammation of the choroid of the anterior part of the eyeball). In 10-45% of cases, the outcome of the disease is severe arthritis.
Polyarthritis, positive for rheumatoid factor, usually develops in older childhood and affects any joints. Occasionally it is accompanied by sacroiliitis. In 50% of cases, children who have had this disease continue to have severe arthritis for the rest of their lives.
60% of children suffering from systemic rheumatoid arthritis are boys. The disease can develop in a child at any age. Any joints can be affected. Rheumatoid factor is absent. In 25% of cases, those who have been ill have severe arthritis for the rest of their lives.
According to the nature of the course, juvenile rheumatoid arthritis can be acute, subacute, chronic and chronic with exacerbations.
Physiotherapy for rheumatoid arthritis here
The signs of juvenile rheumatoid arthritis in children are varied, but the peripheral joints are most often affected. The process begins with the shell and gradually moves to the cartilage. Due to the fact that cells begin to be produced in excess, the joint may change its shape (deform). Next, a layer of cells is formed that covers the surface of the joint and disrupts metabolic processes - this further increases the destruction of tissue. The onset of the disease can be acute or subacute.
In case of acute onset, the following symptoms are noted:
Often juvenile rheumatoid arthritis is accompanied by damage to the spine and temporomandibular joints. Subacute onset is characterized by a less pronounced clinical picture. Function is impaired gradually; if the knee or ankle joint is affected, gait may change, and young children often stop walking. Sometimes there is morning stiffness: after sleep for half an hour there is a limitation in the range of movements.
Articular manifestations in children are typical and account for about 70% of all symptoms of juvenile rheumatoid arthritis. They are usually symmetrical and lead to deformation and fusion of the articular surfaces, which sharply limits mobility. If the pathology of internal organs is added to the damage to the joints, then they speak of the articular-visceral form, which is characterized by the presence of the following symptoms:
In girls, these symptoms are accompanied by uveitis (an inflammatory change in the membranes of the eye), which affects the membranes of the eye. First, lacrimation, photophobia and decreased visual acuity develop, which can ultimately lead to complete blindness.
Typically, juvenile rheumatoid arthritis is characterized by muscle weakness, anemia and pale skin. Vascular damage is also possible, which leads to a deterioration in the blood supply to the extremities; pigmentation or ulcers develop on the skin caused by insufficient oxygen and nutrients. In the area of the elbows and forearms, characteristic nodules can be found; they are painless, sometimes fused to the bone and do not exceed 5 mm in diameter.
During the course of the disease, kidney function also suffers; this is due to the deposition of a special type of protein (amyloid), which clogs the kidney tubules and reduces filtration capacity. 20% of children experience abdominal pain.
In approximately 40% of children, the disease affects 1-2 joints; the other 40% have multiple lesions. In 20% of children, the disease is systemic, that is, it affects the entire body, not just the joints, and is accompanied by an increase in body temperature - then it is called Still's disease.
Inflammation of only a few joints usually occurs in a child younger than 4 years (usually girls) or older than 8 years (usually boys). The child complains of pain, swelling and stiffness of the joints, usually the knees, ankles or elbows. Sometimes there is stiffness and swelling in one or two other joints, such as a single joint in a toe or hand, wrist, or jaw. Symptoms of joint damage may persist or may periodically appear and disappear.
Girls are especially likely to have inflammation of the iris (chronic iridocyclitis), which is often not accompanied by any symptoms and is only detected during an eye examination. Inflammation of the iris can lead to blindness, so the child should be examined. If this condition is detected, immediate treatment is required.
Multiple joint damage can occur in a child of any age, more often in girls than in boys. Joint pain, swelling, and stiffness begin gradually or suddenly. Usually the knees, ankles, wrists and elbows are affected first, followed by both hands, neck, temporomandibular and hip joints. The inflammation is usually symmetrical, that is, the joints of the same name are affected on both sides - for example, both knees or both hip joints.
Systemic juvenile rheumatoid arthritis occurs equally often in boys and girls. Body temperature rises periodically. Temperatures are usually highest in the evening (often 39°C or higher), then quickly return to normal. When the body temperature rises, the child may feel very unwell. For a short time (often in the evening), a rash consisting of flat, pale pink spots appears on the body and upper legs or arms. She migrates and disappears, then returns. The spleen and some lymph nodes are enlarged. Pain, swelling and stiffness in the joints may be the latest symptoms.
Any type of juvenile rheumatoid arthritis may cause growth problems. When the development of the jaw is disrupted, a decrease in jaw size—micrognathia—can develop.
Children with juvenile rheumatoid arthritis rarely develop rheumatoid factor, an antibody that is usually found in the blood of adults with rheumatoid arthritis. Rheumatoid factor is most often found in girls with multiple joint lesions.
Stages of deforming arthrosis here
Complex treatment of rheumatoid arthritis is used, aimed at a positive effect:
When effective and adequate treatment is used, it is possible to stop the progression of the disease, achieve stable remission, and prevent the development of complications.
According to clinical and anatomical characteristics:
1) RA, predominantly articular form with or without eye damage - polyarthritis, oligoarthritis (2-3 joints), monoarthritis;
2) RA, articular-visceral form (with limited inflammation, Still's syndrome, allergic septic syndrome);
3) RA in combination with rheumatism and other BDSTs.
According to clinical and immunological characteristics:
1) the test for rheumatoid factor is positive;
2) test for rheumatoid factor is negative.
According to the course of the disease:
1) rapid progression;
2) slow progression;
3) without noticeable progression. According to the degree of process activity:
1) high (III degree);
2) average (II degree);
3) low (I degree).
According to the x-ray stage of arthritis:
1) periarticular osteoporosis, signs of effusion into the joint cavity, compaction of periarticular tissues, accelerated growth of the epiphyses of the affected joint;
2) the same changes and narrowing of the joint space, isolated bone lesions;
3) widespread osteoporosis, severe osteochondral destruction, dislocations, subluxations, systemic disturbance of bone growth;
4) the same changes and ankylosis.
According to the patient's functional ability:
2) impaired due to the condition of the musculoskeletal system:
— the ability to self-care is preserved;
- the ability to self-service is partially lost;
- the ability to self-service is completely lost;
3) impaired due to the condition of the eyes or internal organs.
Juvenile rheumatoid arthritis is a lifelong disease, but with properly selected therapy and systematic observation by a rheumatologist, long-term remission with a satisfactory quality of life is possible (study, acquisition of secondary and higher education, professional work are possible). With a frequently relapsing course and systemic manifestations of the disease, the prognosis is more pessimistic - disability occurs early, active life is limited.
In most cases, juvenile rheumatoid arthritis begins acutely or subacutely.
- swelling, deformation and local hyperthermia in the area of the affected joints,
- moderate pain at rest and with movement,
- morning stiffness (up to 1 hour or more),
- limited mobility, changes in gait.
- young children become irritable and may stop walking.
- weakness, moderate splenomegaly and lymphadenopathy,
- weight loss, growth retardation,
- lengthening or shortening of limbs.
- eye damage (uveitis, iridocyclitis), a sharp drop in visual acuity.
Complications of juvenile rheumatoid arthritis are secondary amyloidosis of the kidneys, liver, myocardium, intestines, macrophage activation syndrome with a possible fatal outcome, cardiopulmonary failure, and growth retardation.
- acute infection (most often viral, caused by parvovirus B19, Epstein-Barr virus, retroviruses),
- insolation or hypothermia,
- injections of protein drugs.
One of the leading links in the pathogenesis of juvenile rheumatoid arthritis is a congenital or acquired disorder of immunity, leading to the development of autoimmune processes. In response to the influence of the causative factor, modified IgG (autoantigens) are formed, to which autoantibodies are produced - rheumatoid factors.
Diagnosis of juvenile rheumatoid arthritis is based on medical history and examination of the child by a pediatric rheumatologist and pediatric ophthalmologist, laboratory tests:
— presence of RF, antinuclear antibodies,
- radiography and MRI of joints,
— joint puncture (arthrocentesis).
The criteria for juvenile rheumatoid arthritis are: onset before 16 years of age; duration of illness over 6 weeks; the presence of at least 2-3 signs (symmetrical polyarthritis, deformities of small joints of the hands, joint destruction, rheumatoid nodules, RF positivity, positive synovial biopsy data, uveitis).
Treatment of juvenile rheumatoid arthritis is long and complex and begins immediately after diagnosis. During the period of exacerbation, physical activity is limited (running, jumping, active games are excluded), and exposure to the sun is prohibited. Dietary restrictions include salt, proteins, carbohydrates and fats of animal origin, and sweets. It is recommended to eat foods high in vegetable fats, low-fat fermented milk products, fruits, vegetables, and take vitamins B, PP, and C.
— Glucocorticosteroids (short courses)
Juvenile rheumatoid arthritis is a lifelong diagnosis. With adequate treatment and regular monitoring by a rheumatologist, long-term remission without significant deformities and loss of joint function with a satisfactory quality of life (study, professional work) is possible. The risk of exacerbation may persist for many years. A more unfavorable prognosis is with early onset, continuously relapsing course, RF+ polyarthritis and systemic forms of juvenile rheumatoid arthritis, leading to the development of complications, severe limitations of joint movements and disability. To prevent exacerbations of juvenile rheumatoid arthritis, it is necessary to avoid insolation, hypothermia, changing climate zones, limit contact with infectious patients, and exclude preventive vaccinations and the use of immunostimulants.
A close connection with the school where the child is studying is necessary. Weakness is a common symptom that may require changes to a child's school schedule. It is important to continue his studies even during his hospitalization.
Added (21.03.2016, 14:16)
• This variant of JRA is clinically and genetically indistinguishable from RA in adults.
• The disease usually manifests as polyarthritis involving five or more small joints of the hands and feet.
• Fever is rare.
• Diagnosis criteria require the detection of RF in the blood serum in two series of studies performed twice with an interval of 3 months, each of which should consist of three consecutive tests showing a positive result. This strict approach is justified because temporary increases in RF levels can occur during infectious diseases. It must be remembered that an increase in RF concentration is also possible in SLE, some vasculitis, hypergammaglobulinemia and sarcoidosis
• Radiological signs of periostitis and local osteoporosis are noticeable in the early stages of the disease. One third of patients develop severe functional impairment 10-15 years after the onset of the disease.
• Differences from adult RA include slower growth, a tendency for early metacarpal fusion, and formation of DMJ erosion.
• Joint damage can progress rapidly. The development of edema contributes to limitation of mobility and leads to local demineralization of bone tissue and muscle atrophy.
• On the hands, rapprochement of the carpal bones and tendinopathy are often found, leading to the development of classic variants of the “swan neck” deformity of the hand. The first sign of damage to the elbow joint is flexion contracture. Pathological changes in the shoulder joints are also often detected.
• Similar changes are found in the lower extremities: approximation of the tarsal bones, tenosynovitis and bursitis, flexion deformities of the knee joint, flexion deformities and avascular necrosis of the hip joint.
• The cervical spine lesion resembles that of RA (fusion of apophyseal joints, instability and risk of spinal cord compression). Frequent fusion of apophyseal joints in the region of the III-V cervical vertebrae leads to the formation of a rigid area and the occurrence of subluxations of the above and underlying vertebrae. Atlantoaxial subluxation is found in patients with seropositive arthritis and juvenile AS, rarely in seronegative polyarthropathy.
• Damage to the temporomandibular joint (commonly found) leads to decreased jaw growth and micrognathia. After growth is completed, a violation of the closure of the teeth may require surgical intervention.
There are no specific laboratory signs of seronegative polyarthritis. ESR, C-reactive protein concentration, and white blood cell or platelet count may be elevated or normal. A decrease in red blood cell count is not typical for the disease and requires exclusion of an alternative cause.
• Management of the disease requires a traditional approach. Treatment begins with the prescription of one drug from the NSAID group and continues for 4-8 weeks. If ineffective, the drug is replaced with another. The duration of the second course is also 4-8 weeks. The most commonly used medications are ibuprofen, naproxen, and diclofenac.
• If there is no effect from the use of NSAIDs, intra-articular administration of glucocorticoids is prescribed. In addition, they are used when symptoms of eye damage occur. DMARDs are also prescribed for a period of at least 3 months.
Methotrexate is the drug of first choice. For spondyloarthropathy, sulfasalazine can be used. Long-term treatment is required (even after achieving remission - at least 6 months). Research is currently ongoing into the benefits of taking methotrexate for 6-12 months after achieving remission. If methotrexate is intolerant or ineffective, etanercept is used.
JRA seronegative since 1991. Endoprosthetics of both hip joints and knee joints. Nise. Methotrexate 15 mg. Folieva company. Lozap. Omega - 3. Omez.
Juvenile rheumatoid arthritis (JRA) is a chronic disease of unknown etiology (cause), which occurs in children under 16 years of age and affects the joints, and quite often internal organs are involved in the pathological process.
Juvenile rheumatoid arthritis in children
Juvenile rheumatoid arthritis is a heterogeneous disease. There are various variants of it, both in terms of the degree of joint damage and the involvement (or not) of internal organs, each of which has its own clinical manifestations, course characteristics and different outcomes.
If the disease affects internal organs, then they speak of systemic rheumatoid arthritis. Damage to one joint is monoarthritis, from 2 to 4 is oligoarthritis, more than four are polyarthritis.
It should be noted that in addition to rheumatoid arthritis, other persistent chronic arthritis are possible in children, for example: psoriatic arthritis, ankylosing spondylitis, etc., which in their debut (beginning) are similar to JRA, but later often have a more benign course. Their treatment may also differ. In any case, the diagnosis of juvenile rheumatoid arthritis is made when mandatory diagnostic signs characteristic of this disease are identified through clinical and laboratory examination.
This form of the disease was described by pediatrician J.F. Still at the end of the 19th century. The peak incidence occurs between the ages of 2 and 5 years. In most cases, this form of JRA is characterized by an acute onset: the temperature rises above 38°C, all groups of lymph nodes, as well as the liver and spleen, enlarge. A rash appears on the skin, varying in size and shape. At the same time, there is pronounced swelling, redness of a fairly large number of joints, and a change in their appearance. Quite intense pain occurs both in small joints (hands, feet) and large ones (knees, elbows). Moreover, the damage to the joints is symmetrical, i.e. for example, if the wrist joint hurts, then both on the right and left hand. The joints of the cervical spine and mandibular joints are often involved in the process. Morning stiffness is typical: the child has poor control of his arms and legs in the morning, but by lunchtime his mobility improves.
Rash is one of the symptoms of systemic juvenile rheumatoid arthritis (Still's syndrome)
A blood test reveals an increased number of leukocytes, neutrophils, accelerated ESR, anemia, and high values of C-reactive protein.
This is one of the most unfavorable forms of JRA. With adequate treatment, the general manifestations of the disease (fever, lymphadenopathy, etc.) go away, but joint damage steadily progresses, although at different rates for everyone. In 25-30% of children, despite therapy, irreversible changes occur in the joints with complete or partial loss of mobility in them and disability. In half of the patients, renal function is significantly impaired, often accompanied by a complication - renal amyloidosis with the development of chronic renal failure.
Fortunately, this variant of the course of rheumatoid arthritis in children is now becoming less common (no more than 10%).
It is also characterized by damage to internal organs, high fever, skin rash, enlarged lymph nodes, liver and spleen as in Still's syndrome, but arthritis develops much later, which makes early diagnosis of this disease very difficult.
The temperature with this option is very high, rises in the morning, and simultaneously with the rise in temperature, a rash appears on the torso, arms and legs. Its elements are usually small and accumulate in the form of stripes. The kidneys are often affected (protein and red blood cells appear in the urine) and the heart (weakness, palpitations, fatigue and shortness of breath when moving).
Changes in the appearance of joints with systemic rheumatoid arthritis in children (photo) At the onset of the disease, the child may complain of pain in the joints, but swelling, redness and impaired mobility are not detected in them. After about 2-3 years, some patients develop symmetrical arthritis of large joints: hip, knee, ankle. The hands and feet are extremely rarely affected in this form. Currently, with proper treatment, in more than 50% of children, the allergic variant of rheumatoid arthritis ends in recovery, without persistent and irreversible joint changes.
This variant occurs most often in teenage girls and is similar in its symptoms and course to rheumatoid arthritis in adults. Small joints of the hands and/or feet, wrist joints, as well as knee and ankle joints are affected symmetrically on both arms and legs. Morning stiffness lasts from an hour to 3-4 hours, depending on the activity of the process. The joints hurt, swell, their configuration changes, and over time, deformations and ankylosis characteristic of RA appear (complete immobility of the joint). Strength and muscle mass decrease, i.e. muscle atrophy occurs around the diseased joints. Internal organs are not affected in this variant; blood tests usually do not show such strong changes as with Still's syndrome, but ESR and C-reactive protein may increase.
Symmetrical damage to the joints of the hands in rheumatoid polyarthritis
When examining patients with JRA, the so-called rheumatoid factor (RF) must be determined. Variants with positive RF (seropositive rheumatoid arthritis) have a more aggressive course.
It occurs more often in girls at an early age. Usually, pain, swelling and impaired movement first appear in one joint, most often in the right knee, or in 2-4 joints: additionally the ankle and/or wrist. These are favorable forms of JRA: they rarely cause complete destruction of the articular surfaces. Approximately 40% of children eventually develop pain and swelling in other joints, i.e. the disease transforms into polyarthritis, which, however, progresses favorably and does not affect as many joints as in the previous version. Internal organs are usually not affected.
In other children, the disease occurs with periodic exacerbations in one - two - three joints without noticeable progression, the pain syndrome is moderate, mobility is quite well preserved. This seronegative (RF is not detectable) variant responds well to therapy.
Pain and dryness in the eyes may be a precursor to the oligoarthritic form of juvenile rheumatoid arthritis
A common complication of the oligoarthritic form of JRA is eye damage. Children complain of pain, dryness, a feeling of “sand in the eyes”, it becomes difficult for them to look at bright light, and visual acuity decreases. Sometimes eye complaints appear earlier than joint pain.
As we can see, rheumatoid arthritis in children occurs very differently, with different symptoms and activity of autoimmune inflammation. X-ray diagnostics helps doctors differentiate the disease. X-rays of patients with JRA reveal bone loss (osteoporosis), impaired bone growth, and narrowing of joint spaces. In late stages or high activity of the disease, signs of destruction of the articular surfaces and cartilage (cysts and usuria - ulcerations of bones) and their fusion with each other (ankylosis) appear on the radiograph.
If the clinic has the opportunity to conduct special immunological studies, this makes it possible to diagnose JRA and its variants as early as possible and begin treatment on time. Just as in adults, the earlier therapy is started, the greater the chance of preserving joint function and preventing severe complications.
JRA is a systemic disease of connective tissue with predominant damage to the joints of the type of erosive-destructive progressive arthritis. The disease has been known since the mid-nineteenth century and was described by M.V.Cornil back in 1864.
For many years, in the domestic nomenclature and classification, this disease was called “nonspecific infectious polyarthritis.” In recent decades, the term “rheumatoid arthritis” (RA) has become firmly established in medical practice. Variants of its course that occur in childhood are called JRA.
JRA has quite specific features that distinguish this disease from RA in adults. The main reason lies in the fact that articular syndrome in childhood in the early stages of its development in many rheumatic diseases has common clinical and immunological characteristics, which often leads to diagnostic errors. This is facilitated by the physiological characteristics of the growing child’s body in general and the immune system in particular, which during childhood goes through a number of stages of its development and formation.
Currently, all European countries, except Russia, have approved the term “juvenile chronic arthritis” (JCA), which unites almost all chronic inflammatory diseases of the joints in children, including JCA. This terminological restructuring complicates the interpretation of the results of studies conducted in the study of inflammatory diseases of the joints, including in children suffering from JRA.
Many years of experience in studying JRA allows us to believe that this is a heterogeneous group of diseases in which, regardless of the etiology and pathogenetic mechanisms, damage to the structures of the articular apparatus as target organs is always present to one degree or another.
In terms of prevalence, JRA ranks first among inflammatory diseases of the joints, has a clear tendency to develop early disability, and is characterized by the involvement of vital organs (heart, eyes, kidneys, liver, etc.) in the process in some children. This places JRA in the category of exclusively relevant diseases of pediatric rheumatology.
The prevalence of JRA in different regions of the globe varies and ranges from 0.1 to 0.8% of the population. Girls get sick more often.
No single or main etiological factor of JRA has been established, therefore there is a general opinion that JRA is multi-etiological, i.e. heterogeneous disease in its genesis. Factors predisposing to the development of JRA and factors contributing to the implementation of JRA (if there are predisposing factors) are identified. It has been proven that all factors predisposing to the development of JRA have tropism for joint tissues and can persist in them for a long time, causing immune inflammation. From these positions, a number of viruses belonging to the group of “persistent” in the body are discussed. These are mainly DNA and RNA containing viruses (oncornaviruses, retroviruses, etc.), capable of replacing genomic regions on chromosomes, remaining “inactive” for a long time, and only if they are initiated by any provoking factors, perform mutagenic functions. The “initiators” of their action can be frequent diseases that weaken immune mechanisms, hypothermia, insolation, vaccinations, injuries, unfavorable environmental factors (radiation exposure, accumulation of a number of heavy metals, etc.), chronic psycho-emotional stress.
We studied the levels of accumulation of a number of heavy metals and trace elements in children suffering from JRA. It was found that 50% of them had an increased chromium content (3-4 times higher than acceptable levels), and 85% of patients with JRA had a high concentration of cadmium in the blood serum. The degree of increase in these microelements in the blood serum of children suffering from JRA correlated with the levels of their accumulation in the soil and concentration in water at the place of residence of the patients. This fact suggests the involvement of chromium and cadmium in the development of the disease (L.M. Belyaeva, E.V. Voitova, S.M. Korol, 1998; L.M. Belyaeva, E.K. Khrustaleva, 2003).
The role of the Epstein-Barr virus in the genesis of JRA has been established. This virus has a pronounced mutagenic effect and high affinity for immunocompetent substances. The intensively ongoing study of the effects of this virus proves its involvement in the development of many immunological changes in the body, which, with “individual characteristics”, including the presence of a number of markers in the HLA system, creates conditions for the development of severe rheumatic, a number of oncological and hematological diseases.
In terms of the possible etiological factor of JRA, the rubella virus is discussed, which, having a tropism for articular structures, can provoke the development of synovitis. The presence of “minor” primary immunodeficiency states is also discussed: selective IgA deficiency, hypogammaglobulinemia, deficiency of fractions C 2 and C 4 of complement, etc.
A special role is given to the association of the disease with the carriage of a number of antigens of the HLA system (B35; B12; DR1; DR2; DR4; DR5; DW14; DQ2; B27).
In general, JRA is a polygenic predisposing disease. The heterogeneity of the factors that initiate its development in general determines the clinical polymorphism of JRA. Probably, each variant of the course of JRA has its own mechanism of implementation, in which the characteristics of the immunogenetic status play a role, as well as the presence of the persistence of the viral factor in the child’s body. The background may be minor connective tissue dysplasticity syndromes.
Phenotyping of patients with JRA using the HLA antigen system made it possible to associate a number of forms of the disease with the predominant frequency of carriage of one or another antigen. It has been established that the most severe forms of JRA involving internal organs are observed in children who carry DR locus antigens in combination with B35. Carriage of HLA locus antigens B12, B40, B27 is more often associated with predominantly articular forms of JRA; seropositive variants of JRA correlate with the presence of HLA-DR4. It can be considered that the DR locus of the HLA system is involved in the genetic determination of clinical polymorphism of JRA.
The study of phenotypic characteristics according to the HLA system in patients with JRA and their family members opens up prospects for primary and secondary prediction of this serious disease. Despite the fact that families of those examined rarely have several children or close relatives suffering from JRA, the results of studying the haplotypes of HLA histocompatibility antigens make it possible to predict a congenital predisposition to this disease. This, in turn, involves individualization in approaches to organizing lifestyle (sports, additional exercise, nutrition, etc.), hardening, preventive vaccinations, etc.
Literary data regarding the clarification of the HLA phenotype in children with different forms of JRA indicate the possibility of choosing treatment tactics.
In patients with JRA with an association with HLA-B8 and HLA-DR3, severe proteinuria is detected when treated with gold preparations, and in patients with the presence of the HLA-B35 antigen, the effect of steroid therapy is higher. Patients with HLA-DR3 and HLA-A3 haplotypes often have a rapidly progressive course of JRA, despite the treatment, and those who carry the HLA-B5, DR1 and DR2 antigens often experience a better prognosis and a slower progression of erosive and destructive processes in the joints. Most family studies suggest that the presence of the HLA-DR4 haplotype aggravates disease, which is likely due to familial aggregation of joint diseases.
Thus, JRA is a multifactorial disease with a hereditary predisposition, in the genesis of the development of which immunogenetic factors, “persistent viral infections,” adverse environmental influences, and psychoemotional stress play a role. Clinical polymorphism is determined by the etiological heterogeneity of the disease.
It is difficult to look at a patient with rheumatoid arthritis, but the real grief is the discovery of this disease in one of the relatives, especially in children. However, JRA (adolescent or juvenile rheumatoid arthritis) is a very common disease that forever makes a child different from everyone else.
Rheumatoid arthritis refers to chronic inflammation of the joints. It affects approximately 1.3% of the adult population, but also 0.05% of children under 16 years of age are affected by a rare pathology in this age category. Sometimes the course of the disease, especially if it is not treated, leads to a wheelchair at working age.
The causes of the disease may be different. Doctors have still not come to a consensus on what can affect joints at such a young age, because 50% of teenagers suffering from the disease fell ill before the age of 5 years. Among the most common versions are an infection suffered in infancy (mycoplasma, rubella, hepatitis B), various joint injuries, and disruptions in the immune system. When asked whether rheumatoid arthritis is inherited, experts also answer differently. There have been cases when a child from absolutely healthy parents was diagnosed with a severe form of the disease and, conversely, parents suffering from rheumatoid arthritis raised the child without problems with the joints that were inherited by them. But a genetic predisposition to the disease is still not denied, and joints are more often affected in girls than in boys. Some scientists, who put the genetic causes of the disease first, believe that JRA occurs in women of the first degree of kinship with a certain gene.
Among the possible causes of rheumatoid arthritis, doctors also name stress. But the largest number of patients acquired joint pathology as a result of an infection. Studies have shown that there are virus-like inclusions in the cells of the synovial lining of joints affected by rheumatoid arthritis, but they have not yet been isolated. Smoking is believed to contribute to the development of rheumatoid arthritis.
Most often, peripheral joints are affected: knees, ankles, elbows, hips. Symptoms of the early phase of the disease do not differ from the manifestations of a similar disease in adults. The first clinical symptoms are expressed by pain, limitation of motor functions of the limbs, swelling, and slight deformation. The temperature of the skin over the affected joint often rises.
Particular attention should be paid to the nature of the pain. The most intense pain is observed in the second half of the night and in the morning; by the evening it usually dulls. An undoubted companion to night pain is morning stiffness in the joints in 90-100% of patients. Sometimes joint stiffness begins earlier, which is of particular diagnostic value, but most often the course of the disease is acute, when all of the listed symptoms are observed.
The early phase can last up to one year, and then the next stage of the disease begins - the proliferative-exudative phase, when other progressive phenomena are observed: general weakness, loss of body weight, constant increase in body temperature. Examination at this stage shows deep damage to the joints, the nature of rheumatic pain intensifies. Feverish conditions can be aggravated by rashes on different parts of the body, even in places where the joints have not been affected by rheumatoid arthritis.
Juvenile rheumatoid arthritis is also dangerous because it often affects the heart and lungs. The child complains of lack of air, shortness of breath, and has a sharp dry cough. Lymph nodes increase to 4-6 cm, liver and spleen. At this stage, a complete examination of all organ systems of the adolescent is mandatory in order to identify complications. Girls diagnosed with rheumatoid arthritis may have severe vision problems ranging from watery eyes, photophobia, and complete blindness. A large percentage of patients have growth retardation, and sometimes osteoporosis, resulting in a compression fracture of the spine.
Since girls are more often affected by the disease, parents have a logical question about how the course of the disease in the future will affect the reproductive system, whether their daughter will be able to bear and give birth to a healthy child. Rheumatoid arthritis itself in no way affects the ability to have children, because the reproductive system is not subject to changes during the disease. Ovulation is affected mainly by medications, which no patient can do without. When planning pregnancy, the use of drugs is either canceled or minimized for 6 months. Often pregnancy can cause remission, but in the first months after the birth of the child the disease gains momentum. The reasons for this phenomenon have not yet been found.
For effective treatment of the disease, the most important factor is early diagnosis of the disease; in the case of JRA, the situation is complicated by the fact that at an early stage it is difficult to determine its presence, especially if a child under 5 years of age is ill. In addition to clinical manifestations, it is important to pay attention to the baby’s behavior. The facial expression of a patient with rheumatoid arthritis is restless, the child always tries to sit down so as not to touch the joints, and often cries at night, even when there is no exacerbation.
When visiting a hospital, a child with suspected JRA is prescribed a set of examinations, both laboratory and instrumental. Peripheral blood is taken for the content of red blood cells, hemoglobin, platelets, leukocytes, ESR, biochemical and immunological parameters are analyzed, patients undergo electrocardiography and ultrasound of organs and systems that may be damaged due to the disease. X-rays of affected joints are a lifelong companion for patients with this diagnosis. Children with JRA are tested for infections. With constant use of painkillers and hormonal drugs, esophagogastroscopy is prescribed. Vision deserves special attention, and can be preserved with regular slit-lamp examinations by an ophthalmologist.
In any case, all non-steroidal drugs, immunosuppressive and symptomatic drugs cannot stop the destructive process in the joints, but only relieve inflammation, so the disease is treated by two parallel methods: medicinal and non-medicinal.
The following groups of drugs are used: non-steroidal anti-inflammatory drugs, glucocorticoids, immunosuppressants and biological agents. They reduce inflammation and joint pain, but do not prevent joint destruction. In addition, long-term use of drugs entails many side effects, especially for GCs (glucocorticoids). Only a specialist can create an optimal regimen for taking them in order to prevent serious disorders in the body. Prednisolone can be taken orally, intramuscularly and intraarticularly. In any case, patients experience withdrawal syndrome. In any form, the drug is not prescribed to children under 5 years of age, since one of its most irreversible consequences is a slowdown and complete cessation of growth. Other drugs that have proven themselves in the treatment of JRA include methotrexate, cyclosporine A, and leflunomide. They are not as dangerous in terms of side effects and are not as difficult to discontinue as prednisolone.
Basic principles in the treatment of juvenile rheumatoid arthritis:
If you follow the specialist’s recommendations, long-term remission can be achieved in 75% of patients. Non-drug treatment methods are often used during remission to prolong it and improve the patient’s quality of life. Physical therapy, massage, acupuncture, diet, and reducing the load on affected joints through various orthopedic devices can, together with medications, prevent disability and prolong a person’s life. There are a number of sanatoriums in our country where sick children are treated.
One of the diet options for rheumatoid arthritis and its prevention is considered to be a raw mono diet, which reduces the number of leukocytes in the blood. A raw monoeater is much more likely to live a full life if he has rheumatoid arthritis, but there are differing opinions about the practice's application to children. But even if you are afraid to limit your child’s meat intake, you can apply this type of vegetarianism 1-2 times a year and observe the results.
In adulthood, if rheumatoid arthritis progresses, surgery is used to replace the diseased joint with a prosthesis, but this is not possible while the teenager is developing and the skeleton is not yet fully formed.
Prevention of JRA is impossible due to insufficient determination of the etymology of the disease, but in any case it is important to avoid hypothermia, changes in climatic living conditions, frequent infectious diseases, and unnecessary stress. Treatment of viral diseases should be complete, followed by examination for the consequences of the disease.