Symptoms of juvenile chronic arthritis

Features of the course of juvenile chronic arthritis

The main symptom of juvenile chronic arthritis is arthritis. Pathological changes in the joint are characterized by pain, swelling, deformities and restriction of movements, an increase in skin temperature over the joints. Children are most often affected by large and medium joints, in particular, knee, ankle, wrist, elbow, hip, less often - small hand joints. Typical for juvenile rheumatoid arthritis is the defeat of the cervical spine and maxillofacial joints, which leads to underdevelopment of the lower and, in some cases, upper jaw and the formation of the so-called "bird's jaw".

In terms of the number of affected joints, the following variants of articular syndrome are distinguished:

• oligoarthritis (lesion from 1 to 4 joints)
• polyarthritis (lesion of more than 4 joints)
• generalized arthritis (defeat of all joint groups)

A feature of rheumatoid joint damage is a steadily progressing course with the development of persistent strains and contractures. Along with arthritis, children develop a pronounced atrophy of the muscles located proximal to the joint involved in the pathological process, general dystrophy, growth retardation, accelerated growth of the epiphyses of the bones of the affected joints.

Stages of anatomical changes and functional class in children are also defined as in adults with rheumatoid arthritis in accordance with the Steinbroker criteria.

There are 4 anatomical stages:

• I stage - epiphyseal osteoporosis.
• II stage - epiphyseal osteoporosis, cartilage deformation, narrowing of the joint gap, single erosion.
• III stage - destruction of cartilage and bone, formation of bone-cartilaginous erosions, subluxations in the joints.
• IV stage - criteria of the III stage + fibrous or bone ankylosis.

There are 4 functional classes:

• I class - the functional capacity of the joints is preserved.
• II class - the limitation of the functional capacity of joints without limiting the ability to self-service.
• III class - the limitation of the functional capacity of joints is accompanied by a restriction of the ability to self-service.
• IV class - the child does not serve himself, needs outside help, crutches and other adaptations.

The activity of juvenile chronic arthritis in domestic pediatric rheumatology is assessed in accordance with the recommendations of VA. Nasonova and M.G. Astapenko (1989), V.A. Nasonova and N.V. Bunchuk (1997). There are 4 degrees of activity: 0, 1, 2, 3-y.

In determining the activity of the disease, the following indicators are evaluated:

1. Number of joints with exudation.
2. Number of painful joints.
3. Richie's index.
4. Number of joints with restriction of movements.
5. Duration of morning stiffness.
6. Activity of the disease on the analog scale, assessed by the patient or his parents.
7. The number of systemic manifestations.
8. Laboratory activity indicators: ESR, red blood cell count, hemoglobin level, platelet count, leukocyte count, leukocyte formula, concentration of CRP, IgG, IgM, IgA in serum, RF, ANF.

For the evaluation of remission, it is possible to use modified criteria for the clinical remission of rheumatoid arthritis of the American Association of Rheumatologists.

Criteria for the remission of juvenile chronic arthritis:

1. The duration of the morning stiffness does not exceed 15 minutes.
2. Absence of weakness.
3. Lack of pain.
4. Absence of unpleasant sensations in the joint, pain during movement.
5. Absence of soft tissue edema and effusion in the joint.
6. Normal parameters of acute blood proteins.

The condition can be regarded as a remission if there are at least 5 criteria within 2 consecutive months.

Extrarcticular manifestations

Fever

In the articular articular variant of the course of juvenile rheumatoid arthritis, fever is often subfebrile in nature, with the Stille variant - subfebrile and febrile, with allergoseptic variant - febrile, hectic. Fever develops, as a rule, in the morning hours.

With the allergosis the temperature rises are also observed in the day and evening hours, can be accompanied by chills, increased arthralgia, the appearance of a rash, the increase of intoxication. A drop in temperature is often accompanied by torrential sweats. The febrile period with this variant of the course of the disease can last weeks and months, and sometimes years, and often precedes the development of joint syndrome.

Rash

Rash, as a rule, is a manifestation of systemic variants of juvenile rheumatoid arthritis. It wears a fossil, patchy-papular, linear character. In some cases, the rash may be petechial. The rash is not accompanied by itching, it is localized in the area of the joints, on the face, chest, abdomen, back, buttocks and extremities. It is ephemeral in nature, intensified at the height of fever.

Defeat of the heart, serous membranes, lungs and other organs

As a rule, it is observed in systemic versions of juvenile rheumatoid arthritis. It flows like a myo- and / or pericarditis. These and other processes can be observed in isolation and tend to recur. With pronounced exudate pericarditis there is a threat of cardiac tamponade. Acute myopericarditis can also be accompanied by cardiopulmonary insufficiency.

Clinical picture of heart failure in juvenile rheumatoid arthritis: chest pain, in the heart, and in some cases - isolated pain syndrome in epigastrium; dyspnea of mixed type, forced position in bed (child is lighter in sitting position). Subjectively, the child complains of a feeling of lack of air. When attaching pneumonitis or the presence of stagnant phenomena in a small circle of blood circulation, there may be a moist non-productive cough.

On examination: the patient has cyanosis of the nasolabial triangle, lips, terminal phalanges of the fingers; pastosity (or edema) of the shins and feet; work of the wings of the nose and ancillary respiratory muscles (with cardiopulmonary insufficiency); expansion of the boundaries of relative cardiac dullness mainly to the left, deafness of cardiac tones; systolic murmur over almost all valves; pericardial friction noise; tachycardia, which can reach 200 beats per minute; tachypnoe up to 40-50 breaths per minute; Hepatomegaly with insufficiency in the large circle of blood circulation. In the presence of stagnation in the small circle of the circulation of auscultation, a lot of shallow, bubbly, moist wheezing in the basal parts of the lungs is heard.

With recurrent pericarditis, in rare cases, progressing fibrosis is observed in the formation of a "carapaceous" heart. It is this manifestation of the disease that can lead to death. Along with pericarditis in patients with systemic variants of juvenile rheumatoid arthritis, a polyserositis may develop, including pleurisy, less often perihepatitis, perisplenitis and serous peritonitis.

The defeat of the lungs in patients with rheumatoid arthritis is manifested "pneumonitis", which is based on vasculitis of small vessels of the lungs and interstitial inflammatory process. The clinical picture resembles bilateral pneumonia with an abundance of wet wheezes, crepitations, severe shortness of breath, and signs of pulmonary insufficiency.

In rare cases, it is possible to develop fibroeviruyuschey alviolite, which has poor clinical symptoms and is characterized by increasing respiratory insufficiency.

Frequent extraarticular manifestations also include lymphadenopathy, hepato - and / or splenomegaly.

Lymphadenopathy

Lymphadenopathy is characterized by an increase in the lymph nodes of almost all groups, including cubital and even femoral and bicipital. The most pronounced lymphadenopathy in systemic forms of the disease, when the lymph nodes increase to 4-6 cm in diameter. As a rule, the lymph nodes are mobile, painless, not soldered with each other and with the underlying tnani, soft or tightly-elastic consistency. In the presence of other extra-articular manifestations (febrile and hectic fever, rashes, arthralgias and myalgias, hyperleukocytosis with a shift to the left) differential diagnostics with a lymphoproliferative and and a lobate process is required.

Lymphadenopathy develops not only with systemic versions of juvenile rheumatoid arthritis, but also with joint damage, especially when expressed in polyarticular variants of the disease.

Hepatosplenomegaly

Hepatosplenomegaly develops primarily in systemic versions of juvenile rheumatoid arthritis. More often in combination with lymphadenopathy without cardiac damage, serous membranes and lungs in the Still's variant and in combination with other extra-articular manifestations with an allergoseptic variant.

The persistent increase in the size of the liver and spleen, increased density, and the penicomatous organs of patients with systemic variants of juvenile rheumatoid arthritis may indicate the development of secondary amyloidosis.

Eye disease

Typical for young girls with mono- / oligoarthritis. Anterior uveitis develops. The course of uveitis can be acute, subacute and chronic. With acute uveitis, the patient develops an injection of sclera and conjunctiva, photophobia and lacrimation, pain in the eyeball. The process is characterized by damage to the iris and ciliary body, iridocyclitis is formed. However, most often uveitis with juvenile rheumatoid arthritis is subacute and chronic and is detected even with reduced visual acuity. When the chronic course develops corneal dystrophy, angiogenesis in the iris, spikes are formed, which leads to deformity of the pupil and a decrease in its response to light. Develops a clouding of the lens - cataract. Ultimately, visual acuity decreases, and complete blindness and glaucoma can develop.

Uveitis in combination with oligoarthritis can be a manifestation of reactive arthritis, in combination with spinal cord injury - spondyloarthritis.

Growth retardation and osteoporosis

Juvenile rheumatoid arthritis is one of the diseases that adversely affects growth.

Dysplasia is one of the leading among the many extra-articular manifestations of juvenile rheumatoid arthritis. The growth retardation in juvenile rheumatoid arthritis undoubtedly depends on the inflammatory activity of this disease and is especially pronounced in the systemic version of the course. Systemic chronic inflammation causes a general slowdown and arrest of growth, local inflammation leads to increased epiphysic growth and premature closure of growth zones. At the same time, not only the child's growth process itself suffers, but an asymmetry in the development of the body is formed. It manifests itself underdevelopment of the lower and upper jaws, the cessation of bone growth in length. As a consequence, older children retain body proportions that are characteristic of early childhood.

Negative value also has polyarticular damage to the joints, destruction of cartilage and bone tissue, decreased motor activity, amyotrophy, chronic intoxication with the subsequent development of dystrophy, which also inhibits the growth process.

Undoubtedly, an important factor affecting the growth of patients with juvenile rheumatoid arthritis is the development of osteoporosis. With juvenile rheumatoid arthritis, there are two types of osteoporosis - periarticular - in parts of the bone near the affected joints and generalized. Periarticular osteoporosis develops mainly in the epiphyses of bones that form joints. With juvenile rheumatoid arthritis, it begins to manifest early enough and is one of the diagnostic criteria for this disease. Systemic osteoporosis is more common in children with juvenile rheumatoid arthritis than in adults with rheumatoid arthritis. It develops in all parts of the skeleton, mainly in cortical bones, accompanied by a decrease in the concentration of biochemical markers of bone formation (osteocalcin and acid alkaline phosphatase) and bone resorption (tartrate-resistant acid phosphatase). With the development of systemic osteoporosis, there is an increase in the incidence of bone fractures. Reduction of bone mineral density is most rapid in the first years of the disease, and then slows down. Systemic osteoporosis is more common in children with polyarticular articular syndrome. Its severity is directly correlated with laboratory indicators of disease activity (ESR, C-reactive protein, hemoglobin level, platelet count).

The development of osteoporosis is determined by hyperproduction of the activators of resorption: IL-6, TNF-a, IL-1, granulocyte-macrophage colony-stimulating factor. These cytokines on the one hand are pro-inflammatory and play a leading role in the development of acute and chronic inflammation reactions in juvenile rheumatoid arthritis, and on the other, cause the proliferation of synoviocytes and osteoclasts, the synthesis of prostaglandins, collagenase, stromelysin, activation of leukocytes, enzyme synthesis, which leads to development local and systemic osteoporosis. Along with hyperproduction of activators of resorption in patients, there is a deficiency of inhibitors of resorption (IL-4, gamma-interferon, soluble IL-1 receptor).

The risk factors for the development of short stature in patients with juvenile rheumatoid arthritis are: early onset of the disease, systemic variants of juvenile rheumatoid arthritis, polyarticular syndrome, high disease activity, glucocorticoid therapy, and osteoporosis.

Identification of risk factors will allow to predict and possibly prevent the development of such serious complications as nanism, osteoporosis, as well as to develop schemes of differentiated and safe treatment of the disease at early stages.

In general, rheumatoid arthritis in children is characterized by a chronic incontinently progressive course with the development of often lifelong disability. Despite active therapy by the age of 25, 30% of patients who fell ill with juvenile rheumatoid arthritis at an early age remain active. Half of them become disabled. In 48% of patients, severe disability develops within the first 10 years after the onset of the disease. People who suffer from rheumatoid arthritis since childhood are lagging behind in growth. 54% of them have osteoporosis. In 50% of patients, a reconstructive operation on the hip joints was performed by the age of 25. In 54% of adults who fell ill with juvenile rheumatoid arthritis in childhood, sexual disorders are detected. In 50% of the patients there is no family, 70% of women do not have a pregnancy, 73% have no children.

There are several variants of the course of juvenile rheumatoid arthritis: systemic, polyarticular, oligoarticular.

Systemic version of the course of juvenile chronic arthritis

The share of the systemic version of the flow accounts for 10-20% of cases. Develops at any age. Boys and girls get sick with the same frequency. Diagnosis of a systemic version of juvenile rheumatoid arthritis is determined by the presence of arthritis accompanied by fever (or with a previous documented fever) for a minimum of 2 weeks in combination with two or more of the following symptoms:

1. rash;
2. serosite;
3. generalized lymphadenopathy;
4. hepatomegaly and / or splenomegaly.

When diagnosing systemic juvenile rheumatoid arthritis, the presence and severity of systemic manifestations should be assessed. The course of the disease is acute or subacute.

Fever - febrile or hectic, with temperature rises predominantly in the morning, often accompanied by chills. With a drop in temperature, there are torrential sweats.

The rash has a spotty and / or patchy-papular character, linear, is not accompanied by itching, is not persistent, appears and disappears for a short time, is amplified at the height of the fever, localized mainly in the region of the joints, on the face, on the lateral surfaces of the trunk, buttocks and extremities . In some cases, the rash may be urtic or hemorrhagic.

With a systemic version of juvenile rheumatoid arthritis, internal organs are affected.

The defeat of the heart proceeds more often according to the type of myopericarditis. In this case, the patient complains of pain in the region of the heart, in the left shoulder, in the left scapula, in pericarditis in the epigastric region; to a sense of lack of air, palpitations. The child can take a forced position - sitting. On examination, the doctor should pay attention to the presence of cyanosis of the nasolabial triangle, acrocyanosis, pulsation of the heart area, epigastric region. When percussion of the boundaries of relative cardiac dullness, an extension to the left is revealed. With auscultation of the heart, the tones are muffled, a pronounced systolic murmur is heard, often over all the valves; characterized by tachycardia, up to paroxysmal, with pericarditis determine the pericardial friction noise. With recurrent pericarditis develops progressive fibrosis with the formation of a "carapaceous" heart.

The defeat of the lungs can manifest pneumonitis or pleuropneumonitis. The patient complains of a feeling of lack of air, a dry or wet unproductive cough. When examining, you should pay attention to the presence of cyanosis, acrocyanosis, dyspnea, to participate in the act of breathing auxiliary muscles, wings of the nose. At auscultation, listen to abundant small bubbling rales and crepitation in the lower parts of the lungs.

With the development of fibrosing alveolitis, patients complain of rapid fatigue, dyspnea that occurs first with exercise, then at rest; on a dry, unproductive cough. When examined, cyanosis is detected, with auscultation - unstable small bubbling rales. The physician should be aware of the possibility of developing fibrosing alveolitis in juvenile rheumatoid arthritis and treat the patient carefully, since the initial stages are characterized by inconsistency of the dyspnea manifestation with insignificant physical changes in the lungs (weakened breathing).

Polyserositis, as a rule, is manifested by pericarditis, pleurisy, less often by perihepatitis, perisplenitis, serous peritonitis. The defeat of the peritoneum may be accompanied by abdominal pain of a different nature. In juvenile rheumatoid arthritis, polyserositis is characterized by a small amount of fluid in the serous cavities.

With systemic juvenile rheumatoid arthritis, vasculitis can develop. On examination, the doctor should pay attention to the color of the palms and feet. It is possible to develop the palmar, rarely plantar, capillaritis, local angioedema, more often in the brush region, the appearance of cyanotic coloring of the proximal parts of the upper and lower extremities (palms, feet) and the marbling of the skin.

Lymphadenopathy is a frequent sign of a systemic version of juvenile rheumatoid arthritis. It is necessary to assess the size, consistency, mobility of lymph nodes, the presence of pain when they palpate. In most cases, an increase in the lymph nodes of almost all groups up to 4-6 cm in diameter is detected. Lymph nodes, as a rule, are mobile, painless, not soldered together or with the underlying tissues, soft or dense elastic consistency.

In most patients, an increase in the size of the liver, less often the spleen, which are usually painless on palpation, with a sharp edge of a densely elastic consistency is determined.

A systemic version of juvenile rheumatoid arthritis can occur with oligo-, polyarthritis or with delayed joint syndrome.

In the systemic variant with oligoarthritis or delayed articular syndrome, arthritis, as a rule, is symmetrical. Most large joints (knee, hip, ankle) are affected. Exudative changes predominate, later deformations and contractures develop. Almost all patients on average on the 4th year of the disease (and sometimes even earlier) develop a coxite with subsequent aseptic necrosis of the head of the femur. In some cases, the joint syndrome is delayed and develops in a few months, and sometimes even years after the debut of systemic manifestations. The child is bothered by arthralgias and myalgia, which increase at the height of the fever.

In the systemic variant with polyarthritis, from the onset of the disease, a polyarticular or generalized articular syndrome with cervical spine lesions is formed, a predominance of proliferative-exudative changes in joints, rapid development of persistent deformations and contractures, amyotrophy, and malnutrition.

With the systemic version of juvenile rheumatoid arthritis, the following complications can develop:

• cardiopulmonary insufficiency;
• amyloidosis;
• growth retardation (especially pronounced at the onset of the disease in early childhood and polyarticular articular syndrome);
• infectious complications (bacterial sepsis, generalized viral infection);
• macrophage activation syndrome.

The macrophage activation syndrome (or hematopoietic syndrome) is characterized by a sharp deterioration in the condition, hectic fever, multiple organ failure, hemorrhagic rash, mucosal bleeding, convalescence, coma, lymphadenopathy, hepatosplenomegaly, thrombocytopenia, leukopenia, decreased ESR, increased serum triglycerides, transaminase activity, increased fibrinogen content and fibrin degradation products (early preclinical trait), a decrease in the level of coagulation factors th blood (II, VII, X). In the punctate of the bone marrow, a large number of macrophages, phagocytizing hemopoietic cells, are detected. Development of the syndrome of macrophage activation can provoke bacterial, viral (cytomegalovirus, herpes virus) infection, drugs (NSAIDs, gold salts, etc.). With the development of the syndrome of macrophage activation, a lethal outcome is possible.

A polyarticular variant of juvenile chronic arthritis

The share of the polyarticular variant of juvenile rheumatoid arthritis is 30-40% of cases. In all classifications, the polyarticular variant, depending on the presence or absence of the rheumatoid factor, is divided into two subtypes: seropositive and seronegative.

Seropositive for rheumatoid factor subtype is about 30% of cases. It develops at the age of 8-15 years. Often sick girls (80%). This option is regarded as adult rheumatoid arthritis with an early onset. The course of the disease is subacute.

The articular syndrome is characterized by symmetrical polyarthritis with knee, wrist, ankle, and also small joints of hands and feet. Structural changes in the joints develop during the first 6 months of the disease with the formation of ankylosis in the small bones of the wrist by the end of the first year of the disease. In 50% of patients, destructive arthritis develops.

Seronegative for rheumatoid factor subtype is less than 10% of cases. It develops at the age of 1-15 years. Girls are more often ill (90%). The course of the disease is subacute or chronic.

The joint syndrome is characterized by a symmetrical lesion of large and small joints, including temporomandibular joints and the cervical spine. The course of arthritis in the majority of relatively benign, in 10% of patients develop severe destructive changes mainly in the hip and temporomandibular joints. There is a risk of uveitis.

In a number of cases, the disease is accompanied by subfebrile fever and lymphadenopathy.

Complications of the polyarticular variant:

• flexion contractures in the joints;
• severe disability (especially with early onset);
• growth retardation (with early onset of the disease and high activity of juvenile rheumatoid arthritis.

Oligoarticular variant of the course of juvenile chronic arthritis

The share of oligoarticular variant of juvenile rheumatoid arthritis accounts for about 50% of cases. According to the classification of the International League of Rheumatology Associations, oligoarthritis can be persistent and progressive. Persistent oligoarthritis is diagnosed when up to four joints are affected during the entire period of the disease; progressive oligoarthritis - with an increase in the number of affected joints after 6 months of illness. The following criteria are used to characterize arthritis: the age of the onset, the nature of the joint lesion (large or small joints are affected, the joints of the upper or lower extremities are involved, the joint syndrome is symmetrical or asymmetric), the presence of ANF, and the development of uveitis.

According to the criteria of the American College of Rheumatology, the oligoarticular variant is divided into 3 subtypes.

Subtype with an early onset (50% of cases) develops between the ages of 1 year to 5 years. It occurs mainly in girls (85%). For articular syndrome, the knee, ankle, elbow, wrist joints, often asymmetric, are affected. In 25% of patients the course of the joint syndrome is aggressive with the development of destruction in the joints. Iridocyclitis occurs in 30-50% of patients.

Subtype with late onset (10-15% of cases) is most often attributed to the debut of juvenile ankylosing spondylitis. It develops at the age of 8-15 years. Mostly boys are ill (90%). Articular asymmetric syndrome. The joints of mainly lower extremities (heel areas, joints of the feet, hip joints), as well as ileosacral articulations, lumbar spine are affected. Developed enthesopathies. The course of articular syndrome is very aggressive, patients develop rapidly destructive changes (especially in the hip joints) and disability. At 5-10% develops acute iridocyclitis.

The subtype, found among all age groups, is characterized by beginning at the age of 6 years. Girls are more often ill. Articular syndrome occurs, as a rule, benign, with non-severe enthesopathies, without destructive changes in the joints.

Complications of the oligoarticular variant of juvenile rheumatoid arthritis:

• asymmetry of the growth of the limbs in length;
• complications of uveitis (cataract, glaucoma, blindness);
• disability (on the condition of the musculoskeletal system, eyes). In the ILAR classification, there are three more categories of juvenile rheumatoid arthritis.

Enthesitis and arthritis

The category of enteritis arthritis includes arthritis, which is combined with enthesitis or arthritis with two or more of the following criteria: pain in ileosacral articulations; pain in the spine of an inflammatory nature; availability of HLA B27; presence in the family history of anterior uveitis with pain syndrome, spondyloarthropathies or inflammatory bowel disease; anterior uveitis associated with pain syndrome, redness of the eyeball or photophobia. To characterize arthritis, assess the age of the onset, the localization of arthritis (small or large joints are affected), the nature of arthritis (axial, symmetrical or progressing to polyarthritis).

Psoriatic arthritis

The diagnosis of psoriatic arthritis is established in children with psoriasis and arthritis; children with arthritis and family history, weighed down by psoriasis from relatives of the first line of kinship, with dactylitis and other lesions of the nail plate. To characterize arthritis, the following criteria are used: age of onset, the nature of arthritis (symmetrical or asymmetric), the course of arthritis (oligo- or polyarthritis), the presence of ANP, uveitis.

The markers of the unfavorable prognosis of juvenile rheumatoid arthritis

Juvenile rheumatoid arthritis is a disease with an unfavorable prognosis in most patients.

The outcome of the disease is determined by the early appointment of adequate immunosuppressive therapy based on the detection of markers of an unfavorable prognosis in the onset of the disease.

Long-term studies have shown that the traditional treatment of juvenile rheumatoid arthritis with drugs that primarily affect the symptoms of the disease (non-steroidal anti-inflammatory drugs, glucocorticoid hormones, aminoquinoline derivatives) do not prevent the progression of bone-cartilage destruction and disability in most patients.

Long-term studies of the features of the course of juvenile rheumatoid arthritis have shown that some indicators of disease activity are of significant significance and can be regarded as a marker for the prognosis of the aggressive course of juvenile rheumatoid arthritis. Among the main of them are:

• the onset of the disease before the age of 5;
• systemic options for the debut of the disease;
• debut as an oligoarthritis of the first and second type;
• a debut as a seropositive variant of juvenile rheumatoid arthritis;
• rapid (within 6 months) the formation of a symmetrical generalized or polyargicular articular syndrome;
• continuously recurring course of the disease;
• significant persistent increase in ESR, concentration of CRP, IgG and rheumatoid factor in serum;
• the increase in functional deficiency of affected joints with limiting the ability of patients to self-service during the first 6 months after the onset of the disease.

In patients with these markers, the malignant course of juvenile rheumatoid arthritis can already be predicted in the debut.

[1], [2], [3], [4], [5]