Juvenile idiopathic arthritis (JIA) is a group of rheumatic diseases that begin on or before the age of 16. Arthritis, fever, rash, lymphadenopathy, splenomegaly, and iridocyclitis are typical findings of the individual forms, the diagnosis is made clinically. The treatment consists of NSAIDs, intra-articular corticosteroids and of anti-inflammatory drugs that slow down the disease progression (DMARD, disease modifying antirheumatic drugs).
JIA is a rare disease. The cause is unknown, but one can assume a genetic predisposition and an autoimmune process. JIA is different from adult RA (Rheumatoid arthritis (RA)), despite occasional similarities.
Juvenile idiopathic arthritis (JIA) is a group of rheumatic diseases that begin on or before the age of 16. Arthritis, fever, rash, lymphadenopathy, splenomegaly, and iridocyclitis are typical findings of the individual forms, the diagnosis is made clinically. The treatment consists of NSAIDs, intra-articular corticosteroids and of anti-inflammatory drugs that slow down the disease progression (DMARD, disease modifying antirheumatic drugs). JIA is a rare disease. The cause is unknown, but one can assume a genetic predisposition and an autoimmune process. JIA is different from adult RA (Rheumatoid arthritis (RA)), despite occasional similarities. Classification JIA does not designate a single disease, but the term refers to a number of chronic arthritis that occur in children and have certain common characteristics. The current classification of the International League of Associations for Rheumatology differentiated categories of illness due to clinical and laboratory findings. Some of the categories are divided into various shapes. The categories include the following: oligoarticular JIA (persistent or extended) Polyarticular JIA (rheumatoid factor [RF] negative or positive) enthesitis-related arthritis psoriatic JIA Undifferentiated JIA Systemic JIA Many of these categories can include more than just one disease, but they are useful to group children with similar forecasts and targeted for treatment. Even children can sometimes change during the course of their illness to other categories. The oligoarticular JIA is the most common form and affects mostly young girls. It is characterized by involvement of joints ? 4 during the first 6 months of disease. Oligoarticular JIA is further divided into 2 types: persistent (? 4 affected joints) and extended (? 5 affected joints after the first 6 months of disease). Polyarticular JIA is the second most common form. It relates ? 5 joints at the onset of the disease and is divided into two further types: RF negative and RF positive. Typically, young girls are RF negative and have a better prognosis. The RF positive form usually occurs in adolescent girl often similar to adult RA. In both types, the arthritis tends symmetrical performing and limited to small joints. The enthesitis-related arthritis Arthritis connects with enthesitis (painful inflammation at the insertion of tendons and ligaments). She’s with older boys more often, the text of which the classic symptoms of spondyloarthropathy develop such. As ankylosing spondylitis, psoriatic arthritis or reactive arthritis. Arthritis tends to occur in the lower extremities and to be asymmetrical. The HLA-B27 allele is more common in this form of JIA. The psoriatic JIA typically occurs in young girls and psoriasis, dactylitis (swollen fingers), nail pits or a family history of psoriasis in a relative connected first degree. Arthritis is often oligoartikulär. An undifferentiated JIA is diagnosed when patients do not meet any criteria for one of these categories or have criteria for more than one of the categories. Systemic JIA (Still’s disease) is the least common form and is characterized by fever and systemic manifestations. Symptoms and complaints manifestations affect the joints and sometimes the eyes and / or skin; Systemic JIA can affect several organs. In children occur in general joint stiffness, swelling, effusion, pain and sensitivity, but some children also have no pain. Joint symptoms can be symmetrical or asymmetrical, affecting both the large and the small joints. A enthesitis typically causes a sensitivity of the iliac crest and the spine, the greater trochanter of the femur, the patella, and the tuberosity of the Achilles and Plantarfaszieninsertionen. JIA can in some cases seriously affects the growth and development. A Micrognathia (receding chin) due to a premature conclusion of the mandibular epiphyses of the lower jaw, or an imbalance of the leg lengths (usually the affected limb is longer) may occur. The most common eye disease is the iridocyclitis (inflammation of the anterior chamber and the anterior vitreous s uveitis caused by connective tissue disease. Juvenile idiopathic arthritis (JIA, formerly known as juvenile RA)). It is usually asymptomatic, but sometimes blurred vision and can cause miosis. In rare cases, when enthesitis-associated arthritis conjunctival injection, pain and photophobia occurs. The Iridocyclitis can lead to scarring (synechiae) and lead to glaucoma with Keratophathie. The Iridocyclitis occurs most often in the oligoarticular JIA. It develops in nearly 20% of patients, especially, when patients have positive antinuclear antibody (ANA). It can also occur in other forms, but is rare in polyarticular RF-positive JIA and systemic JIA. Lesions are mainly used for psoriatic JIA before, which is characterized by psoriatic skin lesions, dactylitis and / or nail pits, but also in systemic JIA, where a transient rash is typical and often appears in connection with fever. The rash in the systemic JIA can be diffuse and migratory, with urticaria or macular lesions with central clearing systemic abnormalities in the systemic JIA include high fever, rash, splenomegaly, generalized lymphadenopathy (particularly of the axillary lymph nodes) and serositis with pericarditis or pleuritis. These symptoms may precede the development of arthritis. Daily bouts of fever occur mainly afternoon and evening and can last for two weeks. Diagnosis Clinical criteria RF, ANA and HLA-B27 test A JIA should be accepted if occurs in children or adolescents arthritis, iridocyclitis, lymphadenopathy, splenomegaly, skin rash or persistent, daily recurring fever. The diagnosis is primarily clinical. Patients with JIA should be tested for RF, ANA and HLA-B27, as these tests can be helpful in distinguishing between forms. In systemic JIA RF and ANA are not detectable in the rule. In oligoarticular JIA ANA are not present in up to 75% of patients and RF in general. In the polyarticular form of RF is, in some cases, especially in adolescent girls positive. HLA-B27 is more common in enthesitis-associated arthritis. In the diagnosis of iridocyclitis slit-lamp examination should be carried out even if no ocular symptoms persist. Therefore, a patient should undergo newly diagnosed oligoarthritis even every 3-4 months and one patient with polyarticular JIA every 6 months of such eye examination. Forecast Complete remissions occur in 50-70% of treated patients. In RF postitiver polyarticular JIA, the prognosis is less favorable. Treatment drugs that delay progression of the disease (especially methotrexate, etanercept and anakinra) Intra-articular corticosteroid injections NSAIDs Similar to the treatment of RA in adults have DMARD, especially methotrexate and biologics (eg. As etanercept, anakinra) the therapeutic possibilities dramatically improved. The symptoms can be improved with NSAIDs, but they do not change the long-term joint disease. NSAIDs are very useful in enthesitis, z. B. Naproxen 5-10 mg / kg p.o. 2 times daily, ibuprofen 5-10 mg / kg p.o. up to 4 times a day or indomethacin 0,5V1 mg / kg p.o. 3 times a day. Systemic administration of corticosteroids is – apart from the severe systemic disease – avoided if possible. If necessary, they are in the lowest possible dose used (eg. As prednisone 0.0125 to 0.5 mg / kg p.o. spread 4 times a day, or the same total dose in one or two daily doses). Growth inhibition, osteoporosis and osteonecrosis are the main problems of a prolonged corticosteroid therapy in children. Intra-articular corticosteroid injections can be given. The dosage for children depends on the weight. Some children need sedation in intra-articular injection, especially when several joints require injections. Methotrexate is at oligoarticular, psoriasis and polyarticular forms of JIA helpful. The therapy is also monitored as in adults. Bone marrow depression and liver toxicity are monitored by determination of blood count, transaminases and albumin. Occasionally, sulfasalazine is used mainly for suspected ankylosing spondylitis. Etanercept, as in adults, blocked TNF-? and is often WIRSAM; 0.4 mg / kg s.c. (Up to a maximum of 25 mg) can be administered 2 times / week or 50 mg 1 time / week. Anakinra, which inhibits the action of IL-1, is particularly effective in some patients with systemic JIA. Flexion contractures is counteracted by the use of physical therapy, exercise, rails and other aid. Adapted aids improve joint function and reduce unnecessary burdens on inflamed joints. Iridocyclitis is treated with ophthalmic Kortikosteroidtropfen and mydriatics and may be a systemic treatment with methotrexate and anti-TNF require (uveitis caused by connective tissue disease: Juvenile idiopathic arthritis (JIA, formerly known as juvenile RA)). Summary JIA comprises a number of different arthritis in children who clinically and biochemically different from each other. A JIA should be accepted if occurs in children or adolescents arthritis, iridocyclitis, lymphadenopathy, splenomegaly or unexplained fever of more than a few days duration or a rash. JIA is clinically diagnosed; Laboratory tests (z. B. on RF, ANA and HLA-B27) are mainly carried out, therefore, to distinguish between the different forms. Progression of the disease can with methotrexate and / or biologics (z. B. etanercept, anakinra) to be stopped. The symptoms are treated with intra-articular injection of corticosteroids and / or NSAIDs. The Iridocyclitis is treated with Kortikosteroidtropfen and mydriatic.