juvenile rheumatoid disease
Introduction
Introduction to juvenile rheumatoid disease Juveniler heumatoidarthritis (JRA) is a common connective tissue disease in childhood. It is characterized by chronic arthritis and is associated with multiple systemic involvement, including joints, skin, muscle, liver, spleen, Lymph nodes. Younger children often have persistent irregular fever, and their systemic symptoms are more pronounced than joint symptoms. Older or adult patients are more limited to joint symptoms. The clinical manifestations of this disease vary widely and can be divided into different types, so many names, such as Still disease (1897), juvenile rheumatoid repeated hyperthermia (Wissler Fanconi syndrome), juvenile chronic arthritis (JCA), juvenile Rheumatism (juveniler heumatoiddisease) and juvenile arthritis (JA). basic knowledge The proportion of illness: 0.003%--0.005% Susceptible people: children Mode of infection: non-infectious Complications: elbow joint stiffness, muscle atrophy
Cause
The cause of juvenile rheumatoid disease
Cause
It is not completely clear yet. It is generally considered to be related to immunity, infection and heredity in the mechanism of opening the door. It seems to belong to type III allergic reaction, causing connective tissue damage. It is possible that the infection of microorganisms (bacteria, mycoplasma, viruses, etc.) continues to stimulate the body to produce immunoglobulin, and serum IgA, IgM, and IgG are increased. Some patients have elevated anti-nuclear antibody titers. There is a rheumatoid factor in the patient's serum, which is a macroglobulin, ie IgM with a precipitation coefficient of 19S, which can react with denatured IgG to form an immune complex, deposited on the synovial membrane or vessel wall, through the complement system. Activation, and release of granulocytes, large monocytic lysosomes; cause inflammatory tissue damage. The level of complement in the serum and joint synovial fluid decreased, IgM, IgG and immune complexes increased, suggesting that the disease is an immune complex disease. There is also a cellular immune imbalance. B lymphocytes increased in mononuclear cells in the peripheral car; interleukin IL-1 increased, while IL-2 decreased. Also involved in the pathogenesis.
In recent years, it has been found that children with less joint type are associated with histocompatibility antigen HLAB27, and it is believed that chromosomal gene inheritance plays a role.
Pathological change
Mainly joint lesions, chronic non-suppurative synovitis. Early joint periosteal hyperemia, edema, accompanied by lymphocyte and plasma cell infiltration, increased synovial fluid, synovial hyperplasia formed into a villus-like protrusion in the joint cavity, synovitis continues to progress, into the advanced stage, synovial villi hyperplasia spread joint Cartilage, and the formation of vasospasm, cartilage can be absorbed, the subchondral bone is eroded, and then the joint is stiff, deformed or subluxated. Children with rheumatoid arthritis rarely have joint destruction.
Non-specific fibrinous serositis can occur in the pleura, pericardium, and peritoneum. The histological change of rheumatoid rash is subepithelial vasculitis.
Prevention
Juvenile rheumatoid disease prevention
As the cause is unclear, there are currently no effective preventive measures.
The course of the disease can be delayed for several years, and acute attacks and remissions occur alternately, and most of them are relieved by themselves in adulthood. But a few still have a seizure. If arthritis has not healed for many years, it can cause severe joint deformity and movement disorders. This is more common in polyarthritis, older girls with onset and systemic type with polyarthritis. In the case of arthritis, chronic iridocyclitis occurs in girls who develop before the age of 4, causing blindness. Ankylosing spondylitis can be seen in older boys. In general, if treated promptly, 75% of patients will be relieved and their joint function is normal. Only a few have caused lifelong disability. Individual patients died of infection or amyloidosis.
Complication
Juvenile rheumatoid complications Complications elbow joint rigidity and muscle atrophy
Common complications of the disease: joint stiffness, deformity or subluxation.
Mainly joint lesions, chronic non-suppurative synovitis. Early joint periosteal hyperemia, edema, accompanied by lymphocyte and plasma cell infiltration, increased synovial fluid, synovial hyperplasia formed into a villus-like protrusion in the joint cavity, synovitis continues to progress, into the advanced stage, synovial villi hyperplasia spread joint Cartilage, and the formation of vasospasm, cartilage can be absorbed, the subchondral bone is eroded, and then the joint is stiff, deformed or subluxated. Children with rheumatoid arthritis rarely have joint destruction.
Non-specific fibrinous serositis can occur in the pleura, pericardium, and peritoneum. Histological changes in rheumatoid rash are subepithelial vasculitis.
Symptom
Symptoms of Juvenile Rheumatoid Symptoms Common Symptoms Muscular Atrophy Joint Stiffness Relaxation Heatocardial effusion rash chills Hepatic dysfunction Hepatosplenomegaly
The clinical manifestations of this disease are very different. The systemic symptoms of infants and young children mainly include relaxation heat and rash. Larger children may have multiple arthritis or only a few joints. According to the clinical manifestations of the first half of the onset, they can be divided into three. Type, has guiding significance for treatment and prognosis.
1, full body type
The systemic type, also known as Still's disease (in the past, used the name of allergic subsepticemia) is characterized by systemic manifestations. The onset is more acute, and the fever is relaxed. It fluctuates between 36 and 41 °C daily. During the chill, the child is in a weak state. After a few hours of heat withdrawal, the child's activity is as usual. The relaxation heat lasts for weeks or months and naturally relieves, but relapses after weeks or months. The rash is also a typical symptom of this type. Often appearing in high fever, with the rise and fall of body temperature, the rash is usually a round congestive maculopapular rash, about 0.2 ~ 1.0cm size, and can be fused into a piece, distributed in the chest and proximal limbs, most suffering Children with liver, spleen enlargement and swelling around the body, may be associated with mild liver dysfunction, about 1/2 patients with pleurisy and pericarditis, X-ray examination showed pleural thickening and a small amount of pleural effusion, occasionally Interstitial pneumonia changes, pericardial effusion is not much, can occur pericardial friction, pericarditis gradually recover, rarely found constrictive pericarditis, myocardial can be affected, but rare endocarditis, most of this type of children Multiple arthritis occurs at the onset or several months later, both large and small joints Involved, arthritis is often neglected due to systemic symptoms at onset. Some patients have only joint pain, myalgia or transient arthritis. A few children can develop arthritis in a few months or years. There may be mild anemia, white blood cells increased significantly, neutrophils have poisonous granules, and leukemia-like reactions may occur. About one-fourth of this type of sick children eventually have severe arthritis, and it is found that there are Hepatosplenomegaly, serositis, low plasma albumin, and persistent systemic symptoms and platelet elevation 600 × 109 / L after 6 months of openness, prone to devastating arthritis.
2, multiple joint type
This type is characterized by chronic symmetrical polyarthritis, involving 5 joints, especially the involvement of the small toe joints. The incidence of girls is more than that of boys. The onset is slow or sudden, showing joint stiffness, swelling and local fever. Generally, there is very little redness, usually starting from the big joints, such as the knees, ankles, elbows, and gradually involving the small joints. For the fusiform fingers, about 1/2 of the children have cervical vertebrae joint involvement, resulting in limited neck activity, jaw Joint involvement causes difficulty in chewing, a small number of rings, sputum (throat cartilage) arthritis, dumbness and throat wheezing, hip involvement and femoral head destruction in the late stage, dyskinesia, recurrent joint symptoms, lasting for several years The joints are stiff and deformed, and the muscles in the vicinity of the joints are atrophy. This type of systemic symptoms is mild, only low fever, loss of appetite, fatigue, anemia, mild liver, spleen and lymph nodes, but rare pericarditis or iris ciliary Somatic inflammation, about a quarter of children with this type of rheumatoid factor are positive, in the late stage of childhood, arthritis is light and heavy, and eventually more than half of the children with severe arthritis.
3, less joint type
There are no more than four affected joints, mainly large joints. This type can be divided into two types: 1I type: the incidence of women and girls is better than that of boys. The age of onset is more than 3 years old. Knee, ankle and elbow joints are good sites. Some are only involved in a single knee joint, occasionally invading the temporomandibular joint or individual phalangeal joints. If the affected joint does not exceed four in 6 months, it usually does not develop polyarthritis, arthritis can recur, but very Less serious dysfunction, this type of sick children in addition to joint symptoms are mainly chronic iridocyclitis, about half of the patients are involved, early often asymptomatic, can be detected with a slit lamp, mostly after the appearance of arthritis symptoms, Even when arthritis has been static, chronic iridocyclitis can cause blindness. Therefore, patients with oligoarthritis should be regularly examined for slit lamp every 3 to 4 months for early detection and timely treatment. Patients may have no joint pain, low fever, fatigue, mild hepatosplenomegaly and lymphadenopathy and mild anemia. Type 2II: Boys have more onset, and the age of good hair is usually after 8 years old, often with family history, and there may be less at home. Patients with rheumatoid arthritis, ankylosing spondylitis or Reiter's syndrome (including arthritis, urethritis and conjunctivitis) or psoriasis are often affected by major joints of the lower extremities, and hip arthritis and paralysis occur early. Ankle arthritis, heel pain and achilles tendinitis, occasional transient paralysis, wrist, elbow arthritis, after many years, patients often have lumbar ankylosing spondylitis, about 75% of patients with histocompatibility Antigen B27 (HLA-B27), some patients develop acute iridocyclitis, but generally do not cause visual impairment.
Examine
Examination of juvenile rheumatoid disease
The disease lacks specific laboratory tests, often anemia during the active period, leukocytosis (more often between 20,000 and 40,000) and markedly increased erythrocyte sedimentation rate, with white blood cells up to 60,000 and nuclear left shift Increased platelet count, up to 1 million in severe systemic type, decreased plasma albumin, increased 2 and gamma globulin, and most positive C-reactive protein. Most arthritic rheumatoid factor-negative patients are 25% anti-nuclear antibody-positive. Rheumatoid factor positive 75% positive, in the arthritic type I, 60% antinuclear antibody positive, sometimes can find lupus erythematosus cells, rheumatoid factor is an antibody specific for IgG, 19S IgM molecule, can Agglutination-sensitized sheep red blood cells, the agglutination titer is positive at 1:32 or above, juvenile rheumatoid arthritis type, girls with a high onset age (about 8 years old or older), and severe joint symptoms are more common, serum IgG , IgM and IgA increased, normal or increased complement, joint synovial exudate examination: appearance turbid, white blood cells increased, up to 5,000 ~ 80,000 / mm3, mainly polymorphonuclear leukocytes, protein increased, normal sugar Or reduce, IgG IgM increased, reduced complement, bacterial culture negative.
Diagnosis
Diagnosis and identification of juvenile rheumatoid disease
diagnosis
The diagnosis of this disease mainly depends on the clinical manifestations. Any systemic symptoms or arthritis symptoms lasting more than 6 weeks, can exclude other diseases, that is, should consider this disease.
Early cases should be differentiated from acute suppurative infections, osteomyelitis, sepsis, septic arthritis, tuberculosis, leukemia and malignant tumors, traumatic arthritis, viral arthritis, in addition, should be associated with rheumatic fever, systemic lupus erythematosus Compared with rheumatic fever, the joint lesions of this disease are mostly bilaterally symmetrical and relatively fixed. The migration is not as good as rheumatoid arthritis. The incidence of subcutaneous nodules is less, and heart valve disease rarely occurs. Immunodeficiency diseases, especially selective IgA deficiency and congenital latent inherited hypogammaglobulinemia, can be similar to rheumatoid arthritis, should be identified, and must be identified with coxa plana disease However, rheumatoid arthritis rarely starts from the hip joint and is not limited to it.
