Juvenile chronic arthritis and its associated uveitis
Introduction
Introduction to juvenile chronic arthritis and its associated uveitis Juvenile chronic arthritis (JCA) is a common idiopathic arthritis that occurs under the age of 16 and usually involves joints such as knees, ankles, and wrists. Uveitis caused by or associated with this disease is A common and important blinding eye disease for children and adolescents. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: cataract Glaucoma Anterior uveitis
Cause
Juvenile chronic arthritis and its associated uveitis etiology
(1) Causes of the disease
The cause is not fully understood.
(two) pathogenesis
The pathogenesis of juvenile chronic arthritis is not fully understood. Some people think that it may not be an independent disease, but may be a syndrome caused by different causes. The study suggests that there are many factors and mechanisms involved in its occurrence. .
1. Collagen-induced cellular immunity and humoral immune response It has been found that patients have specific antibodies and cellular immune responses against type II collagen, and therefore it is considered to be an autoimmune disease, and the immune response is primary. Whether it is secondary or secondary, it is still unclear.
2. Viral infection may be associated with rubella virus and parvovirus B19 infection. The persistent infection of children with synovial rubella may play an important role in the occurrence of this disease.
3. Specific immunodeficiency may be associated with IgA deficiency, no gamma globulinemia, and hypogammaglobulinemia.
4. Molecular mimicking the position of 106-121 of the amino acid residues of the histone H3 of the yeast is similar to the 303-320 of the amino acid residues of the retinal S antigen. After the current introduction into the body, the immune response may cross-react with the retinal S antigen. , causing uveitis, or making the original uveitis worse or relapse.
5. Genetic factors Numerous studies have found that genetic factors play a role in their occurrence, different types have different genetic background, less joint early onset and HLA-DR5, HLA-DRw8, HLA-DRw6, DQw1, DQw2, HLA- Bw35, HLA-DRw52, HLA-DPw2 and other antigen-related; anti-nuclear antibody-positive uveitis oligoarthritis is associated with HLA-Dw5 and HLA-DR5 antigens, especially with HLA-Bw44, DR5, HLA-Bw35, The DR5 antigen is associated with two haplotypes. Delayed joints and vertebral joints are associated with HLA-B27 antigen; rheumatoid factor positive is associated with HLA-DR4 antigen; systemic and HLA-B8, HLA-Bw35, HLA-Bw44, HLA-B14, HLA-Cw4, HLA-DR4, HLA-DR5, HLA-DR7 and other antigen-related.
Prevention
Juvenile chronic arthritis and its associated uveitis prevention
Controlling the symptoms of juvenile chronic arthritis and timely alleviating the condition can delay the occurrence of eye complications.
Complication
Juvenile chronic arthritis and its associated uveitis complications Complications cataract glaucoma anterior uveitis
Uveitis associated with juvenile chronic arthritis can cause complications such as complicated cataract, banded corneal degeneration, secondary glaucoma, ciliary membrane formation, low intraocular pressure, eyeball atrophy, banded corneal degeneration, and complicated cataract And anterior uveitis often coexist, known as the "triple syndrome" of the disease.
Banded corneal degeneration typically begins at the cornea near the limbus at 3 and 9 o'clock. As the disease progresses and over time, the degenerative zone continues to grow, tapering from the sides to the center, and finally forming a transmembrane. Band turbidity, in some patients, the turbid zone can spontaneously decalcify, forming an irregular transparent zone.
Symptom
Juvenile chronic arthritis and its associated uveal inflammation symptoms Common symptoms Uveitis joint swelling Osteoporosis rash Joint dislocation Macular cystic edema Abdominal pain Nodular pericarditis
1. Systemic and joint performance
(1) Systemic type: Systemic juvenile chronic arthritis can occur at any age below 16 years of age. The proportion of males and females is similar, typically showing late fever (39-40 °C), but the body temperature is normal in the morning, skin spots appear. Rash, increased white blood cells, lymphadenopathy, large liver, some patients may still have splenomegaly, pericarditis, pleurisy, abdominal pain and other manifestations, arthritis is often symmetrical, about 1/4 of patients with devastating arthritis, hand, wrist The joints of the feet, ankles, elbows, knees, hips, shoulders, cervical vertebrae, and jaws can be affected. About 10% of the patients are positive for antinuclear antibodies.
(2) polyarticular type: polyarticular juvenile chronic arthritis is more common in women, male to female ratio of 1:3 ~ 1:4, usually concealed onset, can also be acute onset, can appear low fever, in the middle Degree of liver, splenomegaly and lymphadenopathy, subcutaneous nodules, low appetite, anemia, growth retardation, etc., mostly affected by knees, ankles, wrists and other large joints, but can also affect the small joints of the hands and feet, the affected joints more performance It is swollen, painful during exercise, but rarely red, and chronic recurrent inflammation can cause joint deformities similar to those of human rheumatoid arthritis.
(3) less joint type: less joint type juvenile chronic arthritis is more common in women, male to female ratio is 1:5, these patients with systemic manifestations such as fever, white blood cells increased or not, but may have a rash, The knee joint is more susceptible, but it can also involve the ankle, wrist, knuckle, toe joint, ankle joint and spine. The arthritis is usually characterized by joint swelling, pain during activity, tenderness, etc. Severe cases can be disabling, X-ray examination can be no significant change, soft tissue swelling, osteoporosis near the joint, new bone formation of periosteum, bone erosion, narrowing of cartilage space, bone and joint deformation, etc., about 75% The patient presented with positive antinuclear antibodies.
2. Uveitis
In young patients with chronic arthritis, less joint type is most likely to be associated with uveitis, the incidence rate is as high as 20% to 32%, about 5% of patients with polyarticular type have uveitis, and system type has a higher proportion of uveitis. Less, uveitis occurs more than 1 to 10 years after arthritis, in some patients uveitis can be used as the initial performance.
Uveitis caused by or associated with juvenile chronic arthritis affects both eyes. It is reported that the eyes are affected by 67% to 89%. Both eyes may be affected at the same time, but they may be affected successively, but the interval is generally more than several months. More than a few years.
Although juvenile chondritis associated with chronic arthritis is mostly chronic non-granulomatous iridocyclitis, acute anterior uveitis can also occur, as well as retinal choroiditis, total uveitis, retinal vasculitis In addition, patients may also have optic discitis, dry keratoconjunctivitis, corneal melting, chronic lacrimal gland inflammation, etc., therefore, patients with these eye diseases in juvenile should also exclude or determine the presence of juvenile chronic arthritis.
(1) anterior uveitis: Chronic anterior uveitis associated with juvenile chronic arthritis usually begins to occult, patients may have no symptoms, may have slight redness, discomfort, because these patients are younger, The sensation and presentation ability is not perfect, so some patients' inflammation is found at the time of physical examination or when there is chalk or strabismus. The eye examination is usually without ciliary congestion. KP is dusty or medium-sized, located in the lower corneal endothelium. Occasionally, there is a fat-like KP, a small number of patients may have Koeppe nodules, rarely a serious anterior chamber reaction, occasionally see a severe anterior chamber reaction, and even anterior chamber empyema, but the patient's symptoms are mild.
Repeated episodes of anterior uveitis or persistent inflammation often lead to multiple anterior segmental abnormalities, such as pre-irisal adhesions, posterior iris adhesion, pupillary atresia, pupillary membrane closure, stenosis of the anterior chamber, angle closure, iris neovascularization and Hepatic effusion caused by hemorrhage.
Acute anterior uveitis associated with juvenile chronic arthritis occurs mostly in patients over 10 years of age. HLA-B27 antigen test is mostly positive, rheumatoid factor and anti-nuclear antibody test are mostly negative, and eye redness is typical in patients' eyes. Eye pain, photophobia, tearing, a lot of dusty KP, obvious anterior chamber flash, anterior chamber may have serious reactions such as fibrinous exudate and even anterior chamber empyema.
The course of anterior uveitis is manifested in three types:
1 one continuous type: uveitis lasted for more than 1 year, and the remission period was less than 1 month after treatment. 56.9% of patients with type Iris adhesion, 43.1% with cataract, 35.3% with band corneal degeneration and 11.8 with glaucoma %.
2 multiple recurrence types: multiple recurrent episodes, but complete remission, and the remission period is greater than 1 month, about 50% of patients with this type of complications.
3 single hair style: that is, only one episode, the duration of no more than 1 year, after treatment, uveitis continued to relieve, this type of complications occurred less, only 9%.
(2) posterior uveitis: Although anterior uveitis is a common eye lesion in juvenile chronic arthritis, retinal vasculitis is not uncommon, typical retinal capillary vasculitis, fundus examination without obvious abnormalities, fluorescence Fundus angiography revealed extensive leakage of retinal capillaries, sometimes accompanied by cystoid macular edema.
Examine
Examination of juvenile chronic arthritis and its associated uveitis
There is no specific laboratory test for this disease, but some abnormalities can be found in laboratory tests. In particular, anti-nuclear antibody positive is helpful for diagnosis.
The overall positive rate of antinuclear antibodies in patients with uveitis associated with juvenile chronic arthritis is 40%. The younger the age of onset, the more likely the antinuclear antibody is positive, especially in the oligoarticular uveal In female patients with inflammation, the positive rate of antinuclear antibodies is 63% to 100%, but in older male systemic patients, the positive rate of antinuclear antibodies is lower.
Laboratory tests have also found that patients with active inflammation of the systemic and polyarticular types often have normal red blood cell or hemoglobin anemia, usually moderate anemia, in patients with active disease, especially in systemic patients, There are often white blood cells, and the total number of white blood cells usually reaches 30,000 to 50,000/mm3. The increase is mainly neutrophils. The increase of platelets is related to disease activity. The increase is often a precursor to disease progression. In the acute phase, There is an increase in erythrocyte sedimentation rate, an increase in C-reactive protein, an increase in immunoglobulin levels, and an increase in serum complement levels.
The patient's rheumatoid factor positive rate is low, generally around 10%. White separates one subtype from the patient according to the rheumatoid factor, which is called rheumatoid factor-positive polyarticular type. These patients have a late onset age. Erosion of joints and severe arthritis are prone to occur, but uveitis is less likely to occur.
1. In vivo ultrasound biomicroscopy provides an important tool for understanding the changes of the anterior segment caused by uveitis. According to previous reports, juvenile chronic arthritis mainly consists of chronic anterior uveitis. In vivo ultrasound microscopy can be performed on such patients. Multiple changes were found, such as a large amount of exudate around the iris and around the ciliary body, detachment of the ciliary body, choroidal detachment, atrophy of the ciliary body, narrow or closed anterior chamber, closed pupil membrane, and exudation of the flat part of the ciliary body Etc., there may be a "snow-like" change similar to the middle uveitis. It is not clear whether the lesions found are exactly the same as the snow-like changes of the middle uveitis. If they are the same, they can also be considered as juvenile Chronic arthritis is one of the systemic diseases associated with uveitis.
2. Ultrasound examination is helpful for the determination of vitreous lesions and retinal detachment. This examination can be performed for patients with cataract to evaluate the lesions in the posterior segment of the eye.
3. Fluorescein fundus angiography For patients without significant lens opacity, fluorescein fundus angiography can be performed to evaluate whether the retina and retinal vessels are involved.
Diagnosis
Diagnosis and differentiation of juvenile chronic arthritis and its associated uveitis
The diagnosis of this disease is mainly based on arthritis in people under 16 years of age, uveitis triad (iris ciliary body disease, banded corneal degeneration, complicated cataract) and laboratory tests found that anti-test antibody is positive. diagnosis.
Uveitis, which occurs in people under the age of 16 is a type of disease. In addition to juvenile chronic arthritis, other diseases such as toxoplasmosis, retinoblastoma, sarcoma-like disease, leukemia, etc. can cause uveitis. Identification, especially attention to the identification of retinoblastoma, leukemia and other malignant tumors, they can cause camouflage-like camouflage syndrome, retinoblastoma patients typically have retinal yellow-white bulge mass, vascular dilatation on the surface of the tumor Bleeding, tumor cells spread to the vitreous cavity and anterior chamber can cause vitreous opacity, pseudo anterior chamber empyema, iris nodules and other changes, prone to glaucoma, ultrasound, CT, magnetic resonance and other examinations can be seen intraocular or intraorbital inflammatory Lesions, calcifications, etc., are generally not difficult to identify; in leukemia patients typically manifest as retinal hemorrhage, macular oozing or cotton plaque in the macula, retinal nodular infiltration, retinal vascular occlusion and neovascularization, etc. This is significantly different from anterior uveitis associated with juvenile chronic arthritis.
