Usually, arthritis, manifested by pain, swelling, impaired mobility of the joints - the lot of adults. However, it happens that a child complains about pain in the knee, elbow joints, hands or feet, then this is an alarming signal: it is possible that a child develops rheumatoid arthritis, a serious systemic illness.
Juvenile rheumatoid (idiopathic) arthritis - what kind of disease is it?
Juvenile rheumatoid arthritis (or Juvenile idiopathic arthritis, the name adopted by the international league against rheumatic diseases) is a separate disease that differs from rheumatoid arthritis in adults in both clinical and laboratory features.
Juvenile rheumatoid arthritis (JRA) is a systemic multifactorial disease: its cause is still unknown, but an infection that is often provoked is a factor that apparently causes a violation of the immune system of the body. A certain role is played by genetic predisposition: it is known that in those families where there are cases of rheumatic diseases, children more often develop juvenile idiopathic arthritis.
Juvenile rheumatoid arthritis has an autoimmune nature: during the course of a disease, the body begins an “immune attack” on its own connective tissue. Joints are mainly affected.
But joint damage is not the only clinical manifestation of juvenile rheumatoid arthritis. The disease is very multifaceted, with different manifestations. Sometimes the eyes are affected, up to complete blindness. In the systemic form, when the internal organs are involved in the process, the child develops symptoms such as a rash, prolonged fever, and the joints may be weakly affected.
How can a child suspect rheumatoid arthritis?
The disease can occur after a respiratory infection, trauma, as a result of severe stress experienced, and occasionally after an incorrectly administered vaccination. In some cases, provoking factors can not be identified.
If a child complains of pain in the joints (or only in one joint), and this pain does not go away within 1-2 weeks, this is a reason to consult a rheumatologist.
How is rheumatoid arthritis diagnosed?
Fortunately, the achievement of recent years has been the rapid diagnosis of systemic diseases. Ten years ago, the child had to go around a lot of doctors, and the diagnosis was made already when the patient’s condition became quite difficult.
Now, to establish the diagnosis, consultation of the expert - the rheumatologist and a number of researches is required. It usually takes from one month to one and a half to establish juvenile rheumatoid arthritis, in which case the diagnosis is considered to be made in a timely manner.
With articular form rheumatoid arthritis is established according to the clinical picture: if within 2-3 weeks the child has pain, swelling, limited mobility in the joints, most likely he has juvenile rheumatoid arthritis.
When systemic form the child has symptoms that are characteristic of a variety of diseases accompanied by high fever. Therefore, as a rule, the diagnosis is established by the method of elimination, using laboratory and instrumental methods in a hospital.
Sometimes a child develops short-term arthritis, more often after a viral infection (post-viral arthritis)- as a complication after an infection. In this case, the disease is less dangerous to the health of the child and passes much faster: in a few days, in a week. Your local pediatrician can usually cope with post-viral arthritis.
Another common disease involving joint inflammation is reactive arthritis which develops on the background of an endured intestinal or urogenital infection (yersiniosis, dysentery, salmonellosis, chlamydia.) To identify reactive arthritis requires research on markers of these infections. Treatment with antibiotics will allow you to interrupt this disease.
Sometimes, due to ambiguity of symptoms, the district pediatricians refer the child to the orthopedic surgeon, the physiotherapist for a joint complaint. Unfortunately, this tactic only takes the patient precious time, and prescribed therapy can only worsen his condition. It is important that the child also be seen as soon as possible by a rheumatologist.
When is rheumatoid arthritis treated in a hospital?
The child is indicated to be treated in the hospital in one of three cases:
1. He has a suspicion of juvenile rheumatoid arthritis, it is required to establish a diagnosis and select a therapy,
2. In order to monitor the effectiveness and safety of therapy 1 time in 6-12 months,
3. For the intravenous treatment of high-tech genetically engineered biological preparations.
In rheumatoid arthritis, hospitalization is advisable, as it is necessary:
Exclude other dangerous diseases. Sometimes in systemic form, rheumatoid arthritis is similar in symptoms to serious illnesses such as leukemia and tuberculosis. Our task is to eliminate these serious diseases. To do this, a full examination.
Clarify the diagnosis. For this, the child will have to pass a series of tests: blood for markers of disease activity, rheumatic tests, c-reactive protein, antinuclear factor.
Rate lesions. When the articular form, in the hospital, an ultrasound will quickly be done to the child, an x-ray examination, if necessary, MRI of the joints, depending on the form of the disease.
Ensure continuous monitoring of the child’s condition. Sometimes with rheumatoid arthritis, the child’s condition is very serious, there is a threat to his life. In such a situation, it is necessary that a doctor be present next to the child for 24 hours a day, who monitors all changes in his condition.
How long will the treatment take?
As a rule, this disease requires long-term treatment. We use, like the rest of the world, a multi-step method: we start with a simple and gentle therapy, if it does not help, we move on to the next step, and so on until the treatment gives the proper result.
JRA- autoimmune disease. That is, the body's immune system ceases to distinguish some of the body's own tissues from foreign ones, and it starts against them immune aggression. The task of treatment is to suppress this aggression.
At the first stage - nonsteroidal anti-inflammatory drugs are used that are well known not only to rheumatologists - ibuprofen, diclofenac, meloxicam, and others. This therapy, as a rule, is carried out at the stage of diagnostic search and during exacerbation of pain syndrome. Its purpose is to reduce pain and other manifestations of inflammation. It is important to remember that these drugs do not stop the development of the disease and do not prevent the destruction of the joints, therefore, if it is ineffective, basic drugs are prescribed within 2-3 months.
In the second stage - basic drugs are prescribed - methotrexate or, much less often, other immunosuppressive drugs — Sandimmune neoral, Leflunomide. Methotrexate is the “gold standard of JIA treatment” and is the most widely used worldwide for the treatment of JRA. With a relatively mild course of the disease and certain clinical features, the drug sulfasalazine can be used.
In the third stage, with the ineffectiveness of methotrexate, genetically engineered biological therapy is prescribed - these are drugs that block the main pro-inflammatory substances underlying the development of the disease. Therapy is very expensive and requires constant monitoring.
A few years ago, almost all autoimmune diseases were treated with hormone therapy. Now hormones for the treatment of JRA are used much less frequently and in ways that allow to avoid influencing the development of the organism: when administered intravenously and intraarticularly, hormones are removed from the body within a few hours. For a long time and in the form of oral administration, hormones are prescribed in exceptional cases, with the threat of life.
Is rheumatoid arthritis treatment expensive?
For the patient, no. In the majority of patients, all treatment is carried out within the framework of the OMS policy, patients receive all the necessary preparations. In the case of severe variants of the disease, patients receive high-tech therapy — genetically engineered drugs that the state also provides for free.
Can you cure rheumatoid arthritis completely?
Juvenile rheumatoid arthritis is a chronic disease. However, modern therapy makes it possible to achieve remission, when, receiving drugs, the child’s life will not be limited to anything, about a third of the cases, with early start of modern treatment, there is a chance to achieve even non-drug remission (that is, the child will live a full life without or drugs).
Usually, if within two years after the onset of remission the symptoms of the disease do not return, we gradually cancel the supportive therapy. Currently, up to 95% of children diagnosed with rheumatoid arthritis avoid disability (only 12–15 years ago, only 40% of children did not become disabled).
With a favorable outcome, after achieving remission of the disease, children will be able to dance, play sports and lead a full-fledged lifestyle. It is possible that in adulthood, rheumatoid arthritis will again be felt, change the shape and manifestation - so you should be attentive to your health.
What is juvenile arthritis?
Juvenile arthritis is a disease that develops in children under 16 years of age. With its development in patients, there is a characteristic swelling of the synovial membrane, which occurs due to the inflammatory process. Juvenile arthritis can be classified as an autoimmune disease, with the progression of which the patient’s immune system mistakenly begins the process of self-destruction.
According to the available world statistics, juvenile arthritis is diagnosed when examining young patients in 0.6% of cases.
Numerous studies conducted by experts of world-renowned medical institutions have shown the following:
juvenile arthritis does not develop in children under 2 years of age,
in girls, the disease is diagnosed 2 times more often than in boys,
very often patients treated for juvenile arthritis lose their ability to work at a young age,
Stage 1 olinoarthritis (a form of juvenile arthritis) affects 35–40% of patients,
olinoarthritis stage 2 (juvenile arthritis form) affects 10–15% of male patients who have reached the age of 8 years.
Modern medicine defines the following types of juvenile arthritis:
Systemic arthritis. This form of the disease is often referred to as Still's disease, with the progression of which the vital systems of the human body (heart, organs of the gastrointestinal tract, lymph nodes) are affected. Patients have characteristic symptoms: a rash on the skin, an increase in temperature.
Oligoarthritis. This form of the disease has another name - pauciarticular (juvenile) arthritis. During the first 6 months of oligoarthritis in patients 1-5 joints are affected. In most cases, various pathological processes develop in parallel. Most often, this form of the disease is diagnosed in adolescent girls, and as they mature, it can completely disappear.
Polyarthritis. With the development of this form of the disease in patients, more than 5 joints are affected. Polyarticular arthritis is more commonly diagnosed in girls and can affect: the joints of the lower and upper extremities, the jaw, the neck and neck region.
Arthritis that develops after suffering injuries. Some patients diagnosed with this form of arthritis have suffered from skin diseases for several years. Sometimes with such arthritis, the bones, tendons, spine and joints are affected. Most often, the disease is detected in boys under 8 years of age, who have men with spondylitis in the family.
Juvenile arthritis is classified by clinical and anatomical abilities:
articular arthritis, which affects more than 5 joints (if oligoarthritis develops, up to 4 joints are affected in a patient),
articular-visceral form in which syndromes are detected: Wissler-Fanconi, Still,
a form of arthritis with limited viscerite, in which patients have vital organs (lungs, heart, etc.) affected.
Symptoms of juvenile arthritis
Juvenile arthritis is often asymptomatic, but in some cases it is accompanied by characteristic symptoms.:
pain in the area of the affected joints,
stiffness of movement (usually appears after waking up from sleep),
rash on the skin,
sudden weight loss
change the length of the upper or lower limbs,
eye problems (pain, redness, inflammation, blurred vision),
irritability, frequent mood swings, etc.
Causes of juvenile arthritis
The following factors may be considered to cause the development of juvenile arthritis:
viral and infectious diseases suffered by the patient, which provoke an autoimmune reaction in the body,
genetic predisposition to arthritis,
active functioning of the patient’s immune system, against the background of which destruction of the tissues of the joints occurs,
prolonged exposure to the sun
violation of the schedule of preventive vaccinations,
genetic predisposition of the patient to arthritis, etc.
Many patients undergoing complex treatment of juvenile arthritis can develop various complications:
secondary amyloidosis of the myocardium and organs of the gastrointestinal tract,
macrophage activation syndrome (sometimes fatal),
loss of mobility (full or partial),
disability of the patient, etc.
Diagnosis of juvenile arthritis
Diagnosis of juvenile arthritis begins with the collection of the history of the disease. A narrow-profile specialist - rheumatologist, conducts a personal examination of the patient, learns about his lifestyle, hereditary diseases, bad habits, etc. During the inspection, the specialist palpates the areas of the affected joints. It is mandatory for the doctor to indicate in the patient's medical file all the symptoms of the disease and the patient's complaints.
After the initial examination, the patient is sent for additional diagnostics. To do this, he will have to undergo laboratory and hardware tests:
Clinical and biochemical blood tests (the purpose of the study is to determine the indicators of red blood cells, platelets, leukocytes, etc.).
General urine analysis.
A blood test, the purpose of which is to identify bacteria, the presence of which may indicate infection of the bloodstream.
The analysis performed by the orthopedic surgeon, who are sampled synovial tissue and fluid.
Analysis of bone marrow samples to detect leukemia.
X-ray, during which experts identify fractures and other bone damage.
Computed or magnetic resonance imaging.
Scanning of bone and joint tissues, through which you can detect any changes in their structure.
Testing for the presence of: Lyme disease, various viral infections, the determination of erythrocyte sedimentation rate, the detection of antibodies that provoke the development of arthritis, etc.
During diagnostic activities, patients are specially tested, the purpose of which is to identify antinuclear antibodies. This test shows the autoimmune reaction of the human body, in which self-destruction of immunity occurs.
Modern medicine defines 4 degrees of this disease:
remission stage - 0.
In the case when the patient does not detect pronounced symptoms and signs of the disease, when a juvenile arthritis is detected, the doctor will have to diagnose based on the exclusion of other diseases:
Causes of Juvenile Rheumatoid Arthritis
The causes of juvenile rheumatoid arthritis remain controversial. Much attention is paid to the infectious theory of the occurrence of the disease, since many patients show chronic foci of infections.
A connection between arthritis and diseases of bacterial etiology (streptococcus, staphylococcus, and other microorganisms), viral, and mycoplasma is noted. However, there is no sufficiently convincing data in favor of this theory; therefore, juvenile rheumatoid arthritis is considered a polyetiological disease (i.e. caused by a combination of several factors), and the basis of pathological changes are impaired immune responses (presence of certain types of HLA antigens), increased sensitivity to individual environmental factors.
Also of particular importance are gender, age and ethnicity. The influence of genetic factors on the occurrence of the disease is assumed, since in the presence of rheumatoid arthritis in relatives, the risk of becoming ill is doubled.
When juvenile rheumatoid arthritis occurs, the synovial membrane of the joint is affected first. It is believed that as a result of exposure to some antigen, the body begins to produce modified antibodies, which are perceived by the own immune system as foreign (self-antigens). As a result, the cells of the synovial membrane of the joint synthesize antibodies directed against them. They are called rheumatoid factors. Combined with autoantigens, rheumatoid factors form immune complexes that promote the release of a number of biologically active substances, vascular dilation, platelet migration and leukocyte joint cavity.
These reactions lead to the release of enzymes that destroy articular tissue (proteolytic enzymes) and inflammatory mediators. Moreover, the process is chain and cyclical, the release of new substances contributes to the further synthesis of new autoantigens and rheumatoid factors.
Thus, the following changes occur in the joints:
- synovitis with an increase in the amount of intraarticular fluid,
- degeneration and destruction of articular cartilage,
- fibrosis and sclerosis of the joint capsule, the formation of adhesions,
- formation of bone ankylosis.
Rheumatoid arthritis causes complete immobility in the joint, and leads to disability and disability. In children, the disease leads to impaired bone growth due to changes in the nuclei of ossification (earlier maturation). The occurrence of coxitis (arthritis of the hip joint) often leads to aseptic necrosis of the femoral head.
Immune complexes not only persist in the joint for a long time, but also come through the bloodstream to various organs, where they cause similar changes. They are based on the defeat of small arteries (vasculitis). The following reactions are possible:
- liver damage (amyloidosis, inflammation and sclerosis in portal tracts, dystrophy and necrosis of the parenchyma),
- episcleritis (inflammation of the connective tissue between the conjunctiva and the sclera of the eye),
- appearance of trophic ulcers on the legs,
- heart disease (myocarditis and pericarditis),
- pathology of pleura and lungs (pleurisy, pneumosclerosis, pulmonary arteritis),
- kidney damage (glomerulonephritis or interstitial nephritis, amyloidosis),
- pathology of the gastrointestinal tract (as a result of generalized amyloidosis in many organs).
Symptoms of juvenile rheumatoid arthritis
The initial and often the main manifestation of the disease is arthritis. It is characterized by:
- the appearance of pain and swelling in the area of one joint (characterized by the defeat of large joints, most often - the knee or ankle), and after 1-3 months the other joint is involved in the process from the opposite side (inflammation proceeds symmetrically),
- restriction of movement in the joint, especially characterized by the presence of "morning stiffness"
- forced position: the child spares the inflamed joint because of pain,
- deformity of the joint due to edema, and later - due to the destruction of tissues: the knee joints usually acquire a spherical shape, small (for example, wrist or interphalangeal joints of the fingers) - spindle-shaped,
- “joint noise” may occur during movements that most resemble a “snow crunch”,
- involvement in the process of the auxiliary apparatus of the joint: bursitis and tendovaginitis.
In the active stage of the disease, pain in the joint can be so severe that it causes even a slight touch. Gradually, the described changes are increasing, the limitation of mobility in the joints increases, the surrounding muscles atrophy.
Dystrophy is especially characteristic of the muscles located below the inflamed joint.
The outcome of the inflammatory process is the formation of contractures and deformities leading to dislocations and subluxations of the joint, immobility.
Other symptoms of juvenile rheumatoid arthritis:
- general exhaustion, weakness, anemia,
- fever: moderate to severe, predominantly in the morning, accompanied by sweating,
- pains in muscles and joints, aggravated by high fever,
- spotted and / or spotty-papular rash (in the form of nodules), less often - hemorrhagic (ie, in the form of hemorrhages in the skin) or urticarial (in the form of wheals): it is linear, does not itch, rashes are transient, increase at the height of fever , mainly localized in the joints, on the face, on the lateral surfaces of the body, buttocks and limbs,
- lymphadenopathy: an increase in the lymph nodes of almost all groups up to 4-6 cm in diameter, they are, as a rule, mobile, painless, not welded to each other or to the underlying tissues, soft or densely elastic,
- vasculitis: palmar, less often - plantar, capillary, local angioedema (usually in the area of the hand), cyanosis of the palms and feet), “marble” skin coloration,
- serositis: pericarditis, pleurisy, less often - perihepatitis, perispleitis and serous peritonitis (may be accompanied by abdominal pain of a different nature), a small amount of fluid may be detected in serous cavities,
- enlarged liver and spleen (hepatosplenomegaly), while they are painless, with a sharp edge, a densely elastic consistency,
- damage to internal organs (viscerites).
Depending on the presence or absence of organ damage, various clinical forms of the disease are distinguished.
Stages, flow, functional classes
The course of juvenile rheumatoid arthritis is as follows:
- fast progressive
- moderately progressive,
- slowly progressive.
Slowly progressive course can be characterized by articular form of juvenile rheumatoid arthritis with a lesion of 2-3 joints and lack of activity according to laboratory studies. The joint form with the defeat of a large number of joints and the articular-visceral differ in the rapid progression of the process and early lead to the occurrence of ankylosis and irreversible changes in the internal organs.
With the rapid development of the process, the formation of amyloidosis is possible. This is especially true for allergic syndrome. Most often amyloidosis affects the kidneys. This will be indicated by such changes as proteinuria (the appearance of protein in the urine), an increase in the content of cholesterol and lipoproteins in the blood. Later, other signs of chronic renal failure may join: azotemia (increased urea and uric acid in the blood) and edema.
Amyloidosis can also affect other organs: the intestinal wall, liver, spleen, heart, and blood vessels.
The following degrees of disease activity are distinguished:
- High (III degree).
- Medium (II degree).
- Low (I degree).
- Remission (0 degree).
Remission of juvenile rheumatoid arthritis must meet the following criteria (5 of 6 must be present for at least 2 months):
- duration of morning stiffness diagnosis of the disease
The doctor will suspect the presence of juvenile rheumatoid arthritis on the basis of a characteristic clinical picture and patient examination data. Further, he will appoint the necessary laboratory and instrumental methods of research, consultations of narrow specialists.
Depending on the severity of changes according to the results of laboratory and instrumental methods of research, it is possible to estimate the volume of the pathological process and its activity.
Plan of examination of a child with suspected juvenile rheumatoid arthritis:
- Inspection specialist.
- Laboratory research:
- general and biochemical blood tests,
- test for procalcitonin,
- blood and urine cultures for sterility,
- a smear of pharynx and nose on the microflora,
- bone marrow puncture,
- immunological indicators,
- serological studies on the detection of Salmonella, Shigella, Yersinia, Brucella, Chlamydia, Toxoplasma, Toxocar, Trichinella, as well as respiratory, herpetic viruses, Epstein-Barr virus, Hepatitis B and C viruses, CMV, HIV.
- Instrumental examinations: ECG, EchoCG, ultrasound of the abdominal organs and kidneys, X-ray of the chest, spine and affected joints (according to indications - MRI and scintigraphy of the structures of the musculoskeletal system).
- Consultation of an oculist (with examination of ocular media with a slit lamp), ENT doctor.
The following changes may be found in laboratory tests:
- in general, a blood test: a sharp acceleration of ESR (up to 50-80 mm / h), an increase in the number of leukocytes (especially in allergic syndrome) with an increase in band neutrophils, anemia (with a long course of the disease), thrombocytosis,
- biochemical blood test: increase in serumcoid content, diphenylamine reaction,
- immunological studies: an increase in C-reactive protein, an increase in the content of immunoglobulins of classes M and G in the blood, a decrease in the level of complement, the detection of rheumatoid factor, sometimes - positive ANF,
- special research methods: detection of HLADR4 antigen, HLA A2, HLA B27.
Rheumatoid factor is more often detected in rheumatoid arthritis in adults; it is found in children only in cases. Therefore, according to their immunological characteristics, juvenile rheumatoid arthritis is distinguished:
- with the presence of rheumatoid factor (seropositive variant),
- without rheumatoid factor (seronegative option).
In some patients, laboratory values may be within the normal range.
The degree of activity of juvenile rheumatoid arthritis can be determined by laboratory parameters:
- 0 degree: ESR up to 12 mm / h, C-reactive protein - normal,
- I degree: ESR 13-20 mm / h, C-reactive protein slightly increased ("+"),
- Grade II: ESR 21-39 mm / h, C-reactive protein is moderately elevated (“++”),
- Grade III: ESR of 40 mm / h or more, C-reactive protein is dramatically increased (“+++”, “++++”).
Instrumental diagnostic methods:
- Radiography of the affected joints. According to the data obtained, the following stages of the pathological process are distinguished:
- Stage I: osteoporosis in the region of the epiphyses.
- Stage II: disintegration of the cartilage occurs, the joint gap narrows, isolated erosion appears.
- Stage III: destruction of cartilage and bone tissue occurs, bone and cartilage erosion, subluxations in the joints are formed.
- Stage IV: Stage III criteria + fibrous or bone ankylosis.
In children, gross changes in bone tissue are less common than in adults. Early periods of ossification of the nuclei, an increase in the size of the epiphysis on the affected side are detected.
Often there is a lesion of the cervical spine, as evidenced by the narrowing of the articular fissure of the Atlanto-occipital articulation, a decrease in the height of the vertebral bodies, the merging of the vertebrae C1-WITH3.
The listed structural changes in the joints develop during the first 6 months of the disease with the formation of ankylosis in the small joints of the wrist by the end of the first year of the disease.
- ECG and EchoCG to detect myopericarditis. On the ECG, signs of overloading of the heart, disturbances of myocardial repolarization can be recorded. According to EchoCG: an increase in the left ventricular cavity, a decrease in the ejection fraction, hypokinesia of the posterior wall of the left ventricle and / or interventricular septum, signs of relative insufficiency of the mitral and / or tricuspid valves, increased pressure in the pulmonary artery, separation of pericardial sheets and / or free fluid in pericardial cavity.
- Radiography of the chest: with a lesion of the heart - an increase in the size of the shadow of the heart, increased pulmonary pattern and focal shadows. With the defeat of the lungs, there can be a strengthening and deformation of the lung pattern, tyazhy seals and cellular enlightenments (picture of the “honeycomb” lung).
- Ultrasonography of the abdominal cavity and kidneys reveals possible changes in the internal organs.
The diagnostic criteria for juvenile rheumatoid arthritis are:
- arthritis lasting 3 months or more
- damage to the second joint that occurs after 3 months and later
- symmetry of the lesion of small joints,
- tendosynovitis or bursitis,
- morning stiffness
- specific eye damage,
- rheumatoid nodules
- effusion in the joint cavity.
- osteoporosis, small cystic reorganizations of the bone structure of the epiphyses,
- joint space narrowing, bone erosion, ankylosis of the joints,
- bone growth disorder
- defeat of the cervical spine.
Joint form of juvenile rheumatoid arthritis
Depending on the number of affected joints, there are:
- monoarthritis - one joint is affected, which is rarely observed in juvenile rheumatoid arthritis (usually it is arthritis of the knee joint),
- oligoarthritis - 2-4 joints are involved in the process (most often - knee and hip joints, less often - interphalangeal joints of the toes and hands, metatarsus and metacarpophalangeal);
- polyarthritis - damage to more than 4 joints (all joints of the extremities, cervical spine, sternoclavicular joints, temporomandibular joints).
In the articular form of juvenile rheumatoid arthritis, other symptoms of the disease may be present:
- moderate fever,
- swollen lymph nodes
- vegetative disorders.
The more joints involved in the pathological process, the more pronounced the symptoms of the disease.
The articular form can occur with or without eye damage.
Articular-visceral form of juvenile rheumatoid arthritis (systemic variant of juvenile rheumatoid arthritis)
It is characterized by a more severe course due to the involvement of internal organs in the inflammatory process.The diagnosis of systemic form is made in the presence of arthritis, accompanied by fever for 2 weeks in combination with two or more of the following symptoms:
- generalized lymphadenopathy,
- hepatomegaly and / or splenomegaly.
Depending on the features of the clinical picture, the following are distinguished:
- Still's syndrome
- allergic syndrome (Wissler - Fanconi),
- lesions of individual organs.
- acute onset with fever, severe weakness, swollen lymph nodes, often allergic skin rashes,
- severe polyarthritis with a characteristic involvement in the process of the cervical spine and small joints,
- damage to the liver and spleen,
- muscle atrophy, dystrophy, and anemia develop rapidly; new organs are involved in the process.
This is the most severe form of juvenile rheumatoid arthritis. Because of the sharp pain in the affected joints and weakness, the children are almost immobilized, occupying a forced position. It affects the heart (often with the development of myocarditis or pericarditis), the aorta, the respiratory system and the pleura.
Still's syndrome progresses rapidly and often recurs. In the internal organs after the disease remains fibrosis, and in the joints - deformities and bone ankylosis.
Allergoseptic syndrome (Wissler – Fanconi)
- acute onset with high fever (lasts from 2 weeks to 3 months), chills and sweating, allergic rashes (can be of different types),
- joint pains without any significant changes in them (other signs of arthritis may appear several months later or even years after the onset of the disease),
- heart damage (myopericarditis): pain in the left half of the chest, anxiety, feeling short of breath, shortness of breath, forced position in bed, feeling of heartbeat, pallor or cyanosis (cyanosis) of the skin,
- lung damage (pleuropneumonitis, fibrosing alveolitis): dyspnea and cyanosis, cough (first dry, then wet unproductive), wheezing in the lungs, fatigue.
The defeat of individual organs (viscerites):
- usually characterized by subacute or primary chronic course
- a small number of joints are involved in the process (usually not more than 6), fibrous changes develop early,
- damage to one or more internal organs: pericarditis, pleurisy, damage to the lungs, leading to their fibrosis.
As a variant of viscerate, it is possible to have isolated damage to the eyes (usually in the form of uveitis or iridocyclitis) with or without minimal manifestations of arthritis. The disease can lead to ribbon-like corneal dystrophy, cataracts, leading to reduced vision or blindness.
Non-drug treatment of juvenile rheumatoid arthritis:
- restriction of motor activity in the period of exacerbation, with full immobilization is contraindicated, because contributes to the development of contractures, muscle atrophy, exacerbation of osteoporosis and the development of ankylosis,
- therapeutic exercise to eliminate flexion contractures and restore muscle mass, moderate exercise, swimming and walking,
- running, jumping and active games are contraindicated,
- maintaining a straight posture while walking and sitting, sleeping on a hard mattress and a thin pillow,
- exclusion of psycho-emotional stress, exposure to the sun,
- diet with restriction of carbohydrate and fat intake (protein diet), eating foods with a high content of calcium and vitamin D3 for the prevention of osteoporosis,
- with lesions of the hip joints: traction procedures on the affected limb (after prior consultation of the orthopedist), walking on crutches (in the period of coxitis and aseptic necrosis of the femoral head), the movement of the patient without crutches is contraindicated),
- static (tires, splints, insoles) and dynamic orthoses (light removable devices),
- with severe osteoporosis in the thoracic and lumbar spine - wearing a corset or recliner system, with lesions of the joints of the cervical spine - head holder (soft or hard).
Drug treatment of juvenile rheumatoid arthritis:
- nonsteroidal anti-inflammatory drugs (diclofenac, nimesulide, meloxicam),
- glucocorticosteroids (methylprednisolone, betamethasone, triamcinolone),
- immunosuppressants (methotrexate, cyclosporin, leflunomide),
- genetic engineering biologicals (rituximab, infliximab, sulfasalazine),
- other agents (analgesics, antihistamines, immunoglobulin, antibacterial and antifungal therapy, disaggregants, anticoagulants, fibrinolysis activators, etc.) - according to indications.
The choice of drugs, as well as their dose, the duration of therapy is determined by a qualified specialist, taking into account the child's age, the severity of the disease, as well as individual contraindications.
Prognosis and prevention
With the systemic variant of juvenile rheumatoid arthritis, the prognosis is favorable in half of the children. Remission may occur from several months to several years. But relapse may occur years after a persistent remission. In 1/3 of the patients, there is a continuous relapsing course.
The most unfavorable prognosis for persistent fever with thrombocytosis and long-term systemic hormonal therapy. In this case, severe destructive arthritis, amyloidosis, and severe functional insufficiency often develop.
Juvenile rheumatoid arthritis in case of late diagnosis and therapy can quickly lead to severe disorders in the musculoskeletal system, which leads to disability in childhood.
Starting early, the disease can cause growth retardation and even secondary nanism (dwarfism), which can be a source of mental trauma for a child.
In addition, articular-visceral forms of juvenile rheumatoid arthritis result in complications such as amyloidosis of the kidneys and other internal organs, cardiopulmonary insufficiency, the development of severe infectious complications (bacterial sepsis, generalized viral infections) and macrophage activation syndrome (hemaphagocytic syndrome). The latter is characterized by a sharp deterioration of the condition, severe fever, multiple organ failure, hemorrhagic rash, bleeding of mucous membranes, impaired consciousness (even coma), lymphadenopathy, hepatosplenomegaly, hematological changes (thrombocytopenia, leukopenia, slowing of the EOF).
There is no specific prevention of juvenile rheumatoid arthritis. Common measures include:
- observation of children with altered reactivity,
- rehabilitation of chronic infection foci,
- general recreational activities.
In order to prevent recurrence of juvenile rheumatoid arthritis, follow-up monitoring of patients is carried out.
Juvenile (juvenile) rheumatoid arthritis is a diffuse disease of the connective tissue with a predominant lesion of the joints, which develops in childhood and adolescence. Juvenile rheumatoid arthritis is the most common disease in pediatric rheumatology. According to statistics, it is diagnosed in 0.05-0.6% of young patients worldwide. Typically, the pathology does not appear before the age of two, with 1.5-2 times more common in girls. Juvenile rheumatoid arthritis refers to the disabling rheumatic pathology, often leading to disability at a young age.
Classification of juvenile rheumatoid arthritis
Juvenile rheumatoid arthritis is an independent nosological unit that resembles rheumatoid arthritis in adults, but differs from it in articular and extra-articular manifestations. The classification of the American College of Rheumatology (AKP) identifies 3 variants of juvenile rheumatoid arthritis: systemic, polyarticular, and oligo (pauci) articular (types I and II). Type I oligoarthritis accounts for up to 35–40% of cases of the disease, mostly girls are ill, and debut at the age of 4 years. Type II oligoarthritis is observed in 10–15% of patients, mainly in boys, and the onset is at the age of 8 years.
According to immunological characteristics (the presence of rheumatoid factor), juvenile rheumatoid arthritis is divided into seropositive (RF +) and seronegative (RF-). For clinical and anatomical features are distinguished:
- articular form (with or without uveitis) in the form of polyarthritis with a lesion of more than 5 joints or oligoarthritis with a lesion of 1 to 4 joints,
- articular-visceral formincluding Still's syndrome, Wissler-Fanconi syndrome (allergic),
- limited viscerite form (damage to the heart, lungs, vasculitis and polyserositis).
The course of juvenile rheumatoid arthritis can be slowly, moderately or rapidly progressing. There are 4 degrees of activity of juvenile rheumatoid arthritis (high - III, medium - II, low - I, remission - 0) and 4 classes of disease (I – IV) depending on the degree of dysfunction of the joints.
Diagnosis of juvenile rheumatoid arthritis
Diagnosis of juvenile rheumatoid arthritis is based on anamnesis and examination of a child by a pediatric rheumatologist and pediatric ophthalmologist, laboratory tests (Hb, ESR, presence of RF, antinuclear antibodies), radiography and MRI of the joints, joint puncture (arthrocentesis).
The criteria for juvenile rheumatoid arthritis are: debut up to 16 years, disease duration of more than 6 weeks, at least 2-3 signs (symmetric polyarthritis, deformities of small joints of the hands, joint destruction, rheumatoid nodules, positivity in the Russian Federation, positive data of synovial membrane biopsy, uveitis).
Radiological stage juvenile rheumatoid arthritis is determined by the following characteristics: I - epiphyseal osteoporosis, II - epiphyseal osteoporosis with narrowing of the joint space, single edge defects (Uzury), III - destruction of cartilage and bone, numerous Uzury, subluxation of joints, IV - destruction of cartilage and bone with fibrous or bone ankylosis.
Treatment of juvenile rheumatoid arthritis
Treatment of juvenile rheumatoid arthritis is long and complex, begins immediately after the diagnosis. During the period of exacerbation, motor activity is limited (running, jumping, active games are excluded), and exposure to the sun is prohibited. In nutritional restrictions relate to salt, protein, carbohydrates and fats of animal origin, sweets. It is recommended food with a high content of vegetable fats, dairy products with low fat content, fruits, vegetables, taking vitamins of group B, PP, C.
Drug treatment of juvenile rheumatoid arthritis includes symptomatic (anti-inflammatory) drugs of rapid action and pathogenetic (basic) agents. In the acute period of articular syndrome, NSAIDs are prescribed (diclofenac, naproxen, nimesulide), if necessary, glucocorticosteroids (prednisone, betamethasone) by mouth, topically and intraarticularly or in the form of pulse therapy. Prolonged use of basic immunosuppressant drugs (methotrexate, sulfasalazine) reduces the need for symptomatic drugs, prevents progression, prolongs remission and improves the prognosis of juvenile rheumatoid arthritis.
An important component of the treatment of juvenile rheumatoid arthritis is exercise therapy, massage, physiotherapy (medicinal phonophoresis, mud, paraffin, ozokerite applications, UVA, laser therapy) and treatment with natural factors. With pronounced deformities of the joints and the development of severe ankylosis, joint prosthetics are indicated.
Etiology of juvenile rheumatoid arthritis
Until now, doctors have finally failed to find out the reasons for which juvenile rheumatoid arthritis is progressing in children. The etiology of the disorder is based on the negative influence on the state of the baby by external and internal factors. There are the following reasons why children's arthritis develops:
- genetic factor
- severe viral diseases,
- bacterial infectious lesions,
- growth jumps
- joint injuries of varying complexity,
- hypothermia or overheating of the body,
- complications arising after vaccination, which was carried out during or immediately after recovery from SARS,
- intramuscular or intra-articular administration of protein drugs,
- immune abnormalities of congenital or acquired form.
Such a deviation in a child is not rarely diagnosed, but without timely treatment it can lead to serious and irreversible consequences. In juvenile rheumatoid arthritis, joints, internal organs and vital systems are damaged. A feature of the pathology in adults is that in children the destructive process does not stop only on moving joints.
What types are there?
Juvenile rheumatoid arthritis of the knee joint or other mobile joints is a dangerous and complex disease. When classifying it, different criteria are taken into account. The table presents the main types of pathological process: