Juvenile idiopathic arthritis
The juvenile idiopathic arthritis (abbreviated JIA , older synonyms Juvenile rheumatoid arthritis abbreviated, JRA , Juvenile arthritis , abbreviated JCA ) is a chronic inflammatory disease of the joints ( arthritis ) of the rheumatic form circle in childhood ( juvenile ) of unknown cause ( idiopathic ). It represents a special form of chronic polyarthritis . Like systemic juvenile idiopathic arthritis (also: Morbus Still , after George Frederic Still ) and other juvenile forms of rheumatism, the JIA belongs to the field of pediatric rheumatology - the collective names for these diseases are children's rheumatism or common rheumatism in children . In general, they are subsumed under the term autoimmune diseases .
It is believed that they are "triggered" by external factors such as infectious agents and then take a chronic course in genetically predisposed people. Numerous gene locations are involved in the disposition to disease ( polygenic disease ), different genes predominantly occur for different subtypes, and often other gene locations than in diseases of the rheumatic disease group in adulthood are significantly involved.
The diagnosis is made on the basis of the following symptoms in one or more joints in a child (<16 years of age): (usually tolerable) pain, overheating, rarely redness, swelling, effusion and restriction of movement lasting longer than six weeks without any other recognizable cause ( " Exclusion diagnosis ").
The incidence (annual incidence rate) is given as 5–6 in 100,000 children under 16 years of age. It is estimated that 20-30 out of 100,000 children under the age of 16 have rheumatic disease ( prevalence ). This group of diseases therefore sees most of the patients in pediatric rheumatology consultation hours. The majority of these children have oligoarthritis. If several members of a family are affected, the risk of falling ill also increases ten-fold. Identical twins of sick people are at an even higher risk: In individual cases, the onset of the same subtype of juvenile idiopathic arthritis occurs within six months. It could be shown that in a preselected subgroup (children under 16 years of age who need orthodontic treatment) the prevalence increases to approx. 1: 100 (0.88% from 1024 children).
The first symptoms (even if the disease flares up) are often tiredness, tearfulness and poor performance. In contrast to adults, the main symptoms of joint inflammation (pain, swelling, overheating and restricted mobility) cannot be assumed in all parts for the diagnosis of "arthritis" in children. Smaller children often do not express the pain directly. They change their behavior, let themselves be carried, are tearful during motor activities and seem to develop backwards. The affected joints are held in a relieving position, usually in flexion. If left untreated, this inactivity in an axial misalignment can lead to permanent contractures (shortened tendons with permanent restrictions on movement) and muscle wasting. Furthermore, asymmetrical growth of the joints and neighboring bones can occur. In principle, any joint can be affected. Pressure pain can often be triggered over the affected joints. The pain is usually reported by the children as moderate and tolerable. Joint pain is typically more severe in the morning and after prolonged inactivity (“morning stiffness”) and is aggravated by moving the inflamed joint.
Typically, the oligoarticular forms begin asymmetrically, preferably on the large joints of the legs, and the polyarticular forms symmetrically on the small joints of the hands and feet. The systemic form often initially develops joint pain in the cervical spine and, in the long term, often pronounced destruction of the hip joints. Systemic courses and high disease activity can lead to a slowdown in growth and development, delayed puberty and weight loss.
Classification and symptoms and disease courses of the various subgroups
The classification of the International League of Associations for Rheumatology (ILAR) and the WHO from 1998, which has been further developed from older classifications, is usually used in order to summarize the various forms of progression into as homogeneous subgroups as possible for scientific questions (risk factors, concomitant diseases, complications, prognosis, therapy). This classification has not become generally accepted in everyday clinical practice, but its usefulness is continuously being scientifically evaluated and a new revised version is expected shortly.
It is categorized after six months of illness. In the period from six weeks to six months after the onset of the disease, arthritis remains unclassified. For the classification, the disease manifestation applies up to the sixth month after the onset of the disease, even if the long-term course then shows a different pattern of involvement (e.g. some oligoarthritis turn into polyarthritis in the course of the disease). A distinction is made between the following subgroups based on symptoms and laboratory findings:
- Systemic juvenile idiopathic arthritis (also: Still syndrome, Still disease)
- Polyarthritis, rheumatoid factor positive
- Polyarthritis, rheumatoid factor negative
- Persistent (ie 1–4 joints over the long term> 6 months)
- Extended (i.e.> 4 joints in the long term> 6 months)
- Arthritis with enthesitis
- Psoriatic arthritis
- Other arthritis
- Do not meet the criteria above
- Meet the criteria of several categories
Systemic juvenile idiopathic arthritis (ICD-10: M08.2)
Systemic juvenile idiopathic arthritis, sJIA for short (also: Still syndrome, Still disease) is generally considered to be the most severe form of rheumatism in children and can spread to the entire organism. The disease can be diagnosed according to the ILAR classification if the following criteria are met in a child <16 years of age:
- fever recurring daily for at least two weeks followed by inflammation in one or more joints for the next 6 months and
- one or more of the following criteria:
- a salmon-colored, fickle rash,
- generalized lymph node swelling,
- Liver and / or spleen swelling and
- inflammation of serous skins.
Other causes of fever, a medically confirmed psoriasis in the patient or a first degree relative, arthritis in an HLA-B27- positive boy after the age of 6, ankylosing spondylitis , enthesitis-associated arthritis, sacroiliitis in the case of chronic inflammatory conditions must be excluded Bowel disease , Reiter's syndrome , acute anterior uveitis in a first-degree relative and evidence of rheumatoid factor in two examinations at least 3 months apart.
About 10–20% of the courses of juvenile idiopathic arthritis are assigned to the systemic form. The disease manifests itself mainly between the ages of two and eight, boys and girls are affected roughly equally often.
Symptoms at the beginning
The illness usually begins acutely with a high fever, which occurs mainly in the early morning and evening hours. In between, the body temperature drops below 37 ° C. A fleeting, small-spotted, salmon-colored skin rash occurs primarily on the trunk, upper arms and thighs, which can be provoked by heat or mechanically and is often accompanied by itching. Joint pain typically initially affects the cervical spine with pain when turning the head. Inflammation of the large joints, less often of the small joints, takes weeks to months in half of the patients. If arthritis does not occur within six months, a fever of unknown cause is initially assumed . Further symptoms result from polyserositis with pain when breathing deeply ( pleurisy ), heart symptoms ( pericarditis ) and abdominal pain ( peritonitis ). Discrete accumulations of fluid can be detected in these body cavities by sonography. Generalized lymph node swelling as well as liver and spleen enlargement are typical.
Laboratory tests often show maximum signs of inflammation ( ESR , C-reactive protein , leukocytosis , ferritin ), thrombocytosis > 500 / nl, pronounced hypochromic anemia , increased fibrinogen , complement factors (C3, C4), possibly also GOT and LDH . Autoantibodies typical for other autoimmune diseases ( ANA , ds-DNA-AK , ENA , ANCA ) and signs of haemolysis ( Coombs test negative) are missing .
There are often numerous differential diagnoses that must be carefully ruled out before one can speak of systemic arthritis.
Course of disease
The prognosis is inconsistent. Over the long term, around 40% of patients have only mild arthritis, which can be easily controlled with drug and physical therapy. Systemic signs can appear in bursts, sometimes with years of remission. On the one hand, 20–30% permanent remissions are described, on the other hand there are progressive, relatively therapy-resistant courses with irreversible joint destruction and organ complications.
In the long term, chronic progressive patients often focus on joint involvement with radiologically detectable destructive changes, particularly in the hip joints. In addition to all large joints, the small joints can also be affected. Local or systemic osteoporosis can result from the disease-related immobility. With chronic involvement of the cervical spine, the vertebral bodies can fuse. The combination of a progressive joint inflammation with persistent systemic symptoms, persistent signs of inflammation in the laboratory and platelet counts above 500 / nl is particularly unfavorable prognostically.
Complications: In 5–10% of cases with persistently high disease activity, amyloidosis with irreversible organ damage develops . Another complication is the so-called macrophage activation syndrome , characterized by sudden onset of high fever, enlarged liver and spleen, clouding of consciousness and skin rashes. Stunted growth and even short stature can occur in 20 to 41 percent of cases. Advances in immunosuppressive therapy have significantly reduced the frequency of lethal complications. If the total mortality of juvenile idiopathic arthritis is less than 1%, however, more than half are due to the systemic form.
Juvenile polyarthritis, rheumatoid factor positive (ICD-10: M08.0)
This sub-form can be diagnosed according to the ILAR classification if the following two criteria are met: 1. Five or more inflamed joints during the first six months of the disease and 2. evidence of positive rheumatoid factors at least twice with an interval of at least three months.
Among other things, (i) a medically confirmed psoriasis in the patient or a first-degree relative, (ii) arthritis in an HLA-B27-positive boy after the age of six, (iii) ankylosing spondylitis, enthesitis-associated arthritis must be excluded , Sacroiliitis in inflammatory bowel disease, Reiter's syndrome, acute anterior uveiitis in a first-degree relative, and (iiii) signs of systemic arthritis.
5-10% of patients with juvenile idiopathic arthritis belong to this subgroup. It affects about 90% girls, the time of manifestation is mainly around the 7th – 13th centuries. Age.
Symptoms at the beginning
The disease is similar to rheumatoid arthritis in adults . The joints are affected at an early stage, typically symmetrically on the wrists , fingers and toes . In principle, any joint can be affected and significant functional, sometimes irreversible restrictions can occur within months. As in the adult form, subcutaneous rheumatic nodules are observed on the extensor sides of the extremities . These can also occur in internal organs.
Rheumatoid factor-positive polyarthritis can be accompanied by vasculitis of the small and medium-sized arteries and involvement of internal organs. Patients with active polyarthritis also have general symptoms such as stagnation of growth, delay in growth and development, subfebrile temperatures, reduced general condition, decreased performance, weight loss, emotional lability, lymph node swelling and pain and mild hepatosplenomegaly.
The diagnosis requires laboratory proof of rheumatoid factors in at least two examinations every three months.
Course of disease
The disease often progresses rapidly within a few months. The severity of the course can be estimated based on the level of rheumatoid factors and the inflammatory activity ( C-reactive protein , ESR ). Pronounced, radiologically visible, bony lesions with progressive movement restrictions , subluxations and joint misalignments can develop within a few years . This requires early, intensive drug and physiotherapy therapy. Then, if necessary, repair processes can also be achieved on destroyed joints.
The occurrence of systemic symptoms such as vascultitis is prognostically unfavorable and indicates the involvement of internal organs. A pericarditis or polyserositis contrast, developed primarily in the Still's disease . The iridocyclitis (inflammation of the iris of the eye) is much less common in the polyarticular course forms than in the juvenile Oligoarthritiden typically does not lead to permanent eye damage.
Juvenile polyarthritis, rheumatoid factor negative (ICD-10: M08.3)
This sub-form can be diagnosed according to the classification of the ILAR if the following criterion is met: Inflammation of more than five joints within the first six months of the disease.
Among other things, a medically confirmed psoriasis in the patient or a relative of the first degree, arthritis in an HLA-B27-positive boy after the age of six, ankylosing spondylitis, enthesitis-associated arthritis, sacroiliitis in the case of inflammatory bowel disease, equestrian disease must be excluded. Syndrome, acute anterior uveiitis in a first-degree relative, evidence of rheumatoid factor in two examinations at least three months apart, and signs of systemic arthritis.
15-25% of patients with juvenile idiopathic arthritis belong to this subgroup. The maximum age is 2–16 years, around 80% of which are girls.
Symptoms at the beginning
The diagnosis is often preceded by a prolonged period of failure to thrive, weight loss and subfebrile temperatures for months. The joints usually show only moderate swelling, effusion and slight overheating. However, there is often a pronounced restriction of movement with permanent functional deficits. Typically, the wrists, finger joints, and toe joints are affected symmetrically. All large joints can also be affected, including the temporomandibular joints and the cervical spine.
Course of disease
In this form, joint destruction occurs significantly less often and later than in the rheumatoid factor-positive (adult) form, in which these become visible after a few months. Nevertheless, typical misalignments of the wrists ("ulnar deviation", away from the side of the thumb) and fingers (flexion contractures of the proximal finger joints) develop. Radiologically, a pronounced osteoporosis of the affected joints can be seen particularly close to the joints. Furthermore, there is irregular growth: While inflammatory joints often temporarily grow faster, in connection with their training there is a long-term regression of the muscles with reduced growth. In addition, if the growth joint closes prematurely, a permanent growth standstill can occur. The prognosis is functionally unfavorable if the diagnosis is made later and in the case of advanced joint contractures and pronounced axial misalignments. Normal function can only be regained in 10% of these patients. Early diagnosis can significantly improve the functional prognosis with consistent drug and physical therapy as well as the supply of aids.
Juvenile idiopathic oligoarthritis (ICD-10: M08.4)
This sub-form can be diagnosed according to the ILAR classification if the following criterion is met: Inflammation of 1 to 4 joints within the first six months of the disease. If five joints or more are affected after the first six months, this is also referred to as extended oligoarthritis.
Among other things, a medically confirmed psoriasis in the patient or a relative of the 1st degree, arthritis in an HLA-B27-positive boy after the age of 6, ankylosing spondylitis, enthesitis-associated arthritis, sacroiliitis in inflammatory bowel disease, Reiter must be excluded Syndrome, acute anterior uveiitis in a first-degree relative, evidence of rheumatoid factor in two examinations at least three months apart, and signs of systemic arthritis.
Approximately 25–35% of children with juvenile idiopathic arthritis have simple oligoarthritis and approximately 10–20% have advanced oligoarthritis. Juvenile idiopathic oligoarthritis of the simple form begins around the age of two to eight and affects 65% of girls; the extended form begins a little earlier around the age of 18 to six and affects 80% of girls. The disease does not occur in the first six months of life. Children of black African origin are less affected than white children.
Symptoms at the beginning, diagnosis
The parents report motor regressions, swelling of a knee, limping or pain when changing diapers. The examination can be difficult because of the child's defenses and the age-appropriate layer of fat over the joints. The knee is most commonly affected, sometimes the only joint, followed by the ankle and elbow joints. Small finger joints are rare and the hip joint is almost never affected. The affected joints are usually swollen and often overheated, but usually not red. There is often an effusion. The painful restriction of movement that is usually present can only be found if one tries to move the affected joint with the full range of motion appropriate to the age. The affected joints are usually surprisingly less painful than in adults with arthritis.
In 20% of the cases it comes to a mostly chronic course iridocyclitis (inflammation of the iris of the eye). At the beginning this is symptom-free or with few symptoms. The ophthalmologist must actively and repeatedly look for her with the slit lamp , as untreated she can lead to blindness. Risk factors are: female gender, early onset of disease, evidence of ANA , duration of arthritis less than four years.
In the laboratory tests , the inflammation values ( C-reactive protein , ESR ) can be increased or normal. With an ultrasound you can possibly detect effusion and swelling of the soft tissue structures around the affected joint, in some cases magnetic resonance imaging with gadolinium contrast medium can be helpful. The bone scan is rarely used.
Since the symptoms are unspecific, various other diseases must be excluded depending on the duration of the arthritis (including injuries, cancer, osteomyelitis , bacterial joint infections, Henoch-Schönlein purpura , Coxitis fugax , Lyme arthritis , acute rheumatic fever ...). This is made possible by further laboratory tests, a joint puncture with examination of effusion fluid and possibly a bone marrow aspiration. Since oligoarthritis, like other juvenile idiopathic arthritis, is a diagnosis of exclusion, the diagnosis must be re-examined whenever new aspects or manifestations arise.
Course of disease
In the majority of cases, the arthritis disappears as the disease progresses. If it is possible to keep the joint away from permanent damage, the child can emerge from the disease without hindrance. In over 30% of the cases, complete healing can be achieved with consistent therapy. The majority of children go into remissions after months or years, although (mostly mild) relapses are possible many years later.
Without therapy, the inflammation can lead to a growth advantage in the affected leg, which is compensated for by increased knee flexion. Muscle wasting of the anterior thigh muscles develops as well as an axial misalignment of the shinbone to the rear as a result of the unbalanced muscle tension in the hollow of the knee. Ultimately, the entire leg is incorrectly loaded, which is why, without therapy, the neighboring joints are damaged even if the knee joint is exclusively affected, and impaired motor development is to be expected.
The prognosis worsens if the therapy is delayed and if polyarthritis (in 30–50% in the course) or iridocyclitis (in over 30% in the course) occurs. A persistently high ESR can indicate a poor prognosis. However, the prognosis of polyarthritis as extended oligoarthritis is still better than that of the primary polyarthritis.
Late complications of iridocyclitis can include cataract (clouding of the lens), glaucoma (increase in intraocular pressure), phthisis bulbi (shrinkage and atrophy of the eyeball), reduced visual acuity (impairment of visual acuity) or blindness (previously in 15–20% of untreated patients, today in 1 % of professionally treated patients). A manifestation of the second eye is observed in 70% already in the first year of illness.
The other manifestations outside the joints in this form are mostly moderate (anemia, lymph node swelling, subfebrile temperatures) and correlate with the inflammatory activity.
Juvenile idiopathic arthritis with enthesitis (ICD-10: M08.8)
A enthesitis or enthesopathy is an inflammation or pain of ligaments and tendons and joint inflammation or pain distinguishable. Typically, the complaints are at the attachment points of the Achilles tendon (on the heel), the kneecap, the shin, on the extensor and flexor sides of the feet and hands, on the iliac crest, on the coccyx, and more rarely on the chest.
This sub-form of juvenile idiopathic arthritis can be diagnosed according to the classification of the ILAR if the following criteria are met: 1.) inflammation of one or more joints and 2.) tendon or ligament inflammation. If the latter is absent, two of the following five criteria must be met for the diagnosis in addition to joint inflammation: a.) Pain at the transition between the pelvis and lumbar spine, inflammatory spine pain, b.) Presence of the genetic histocompatibility characteristic HLA-B27 , c.) Arthritis onset a boy older than 6 years, d.) anterior choroidal inflammation ( uveitis ) of the eye with pain, redness or photophobia, or e.) the presence of one of the following diseases in a first-degree relative: (i) anterior choroidal inflammation (uveitis) of the eye with pain, reddening or photophobia, (ii) ankylosing spondylitis , (iii) arthritis with enthesitis, (iiii) inflammation of the pelvic-spinal column junction with a chronic inflammatory bowel disease.
Among other things, (i) a medically confirmed psoriasis in the patient or a first degree relative, (ii) evidence of rheumatoid factor in two examinations at least three months apart and (iii) signs of systemic arthritis must be excluded.
This subgroup was introduced in the classification of the International League of Associations of Rheumatology in order to be able to summarize here, based on modified criteria, most of the children of the late form of juvenile idiopathic oligoarthritis listed earlier and also children with what was formerly called juvenile spondyloarthropathy and against psoriasis - To differentiate arthritis by classification. Mostly because of similar genetic risk factors, it is believed that these disorders are related.
Approximately 5 to 10% of children of juvenile idiopathic arthritis can be classified into this subgroup. The age of onset is rather high at 9 to 13 years. Around 20% of girls are affected.
Typically there is pain at the attachment points of the Achilles tendon on the heel, the tendon attachments of the kneecap, the shinbone, the extensor sides of the extremities and the pelvis or deep back. These are particularly pronounced at night and in the early morning hours as well as during physical exertion and lead to significant functional impairments and incorrect posture (protective posture of the spine with an upright position of the lumbar spine and hunched back, incorrect loading of the foot). Joint inflammation occurs primarily on the legs with asymmetrical involvement of large and small joints. It is not uncommon for an entire foot to be affected by inflammation. Often the shoulders and jaw joints are also affected. The involvement of the pelvic-lumbar vertebra transition and the spine usually becomes clear during the course (transition to ankylosing spondylarthritis or ankylosing spondylitis). An exclusive infestation of the spine is very rare in childhood.
The laboratory values usually show an accelerated ESR and an increased C-reactive protein in the disease flare -up . The parameters can slowly decrease over the course of the disease, despite persistent inflammation. The genetic marker HLA-B27 can be detected in around 75% of affected children (occurrence in the normal population: around 8%).
Juvenile psoriatic arthritis (ICD-10: L40.5)
This sub-form can be diagnosed according to the ILAR classification if the following two criteria are met: joint inflammation and psoriasis (psoriasis). If the patient himself does not (yet?) Have psoriasis, it is sufficient if two of the following three criteria are met in addition to the arthritis: a.) Medically confirmed psoriasis in a first-degree relative b.) Inflammation of all joints of a finger or c.) or nail symptoms (peeling or stippling) in the patient.
Arthritis in an HLA-B27-positive boy after the age of six, ankylosing spondylitis, enthesitis-associated arthritis, sacroiliitis in inflammatory bowel disease, Reiter's syndrome, acute anterior uveiitis in a first-degree relative, rheumatoid factor detection in two must be excluded Check-ups at least three months apart for signs of systemic arthritis.
Approx. 5-10% of children of juvenile idiopathic arthritis can be classified in this subgroup. The age of onset is around 6-14 years. Around 65% of girls are affected.
A psoriasis can (psoriasis) of the joint manifestation preceded by several years or even follow. Both occur simultaneously in only 10% of patients. Typical locations for the first manifestation of psoriasis are the hairline, the extensor surfaces of the joints that are exposed to mechanical stress, the umbilical region and the region around the anus. There are also (sometimes discreet) changes to the nails, especially spotted nails. Nail detachment (onycholysis) is atypical for children and should be differentiated from a fungal disease. Infestation of all joints of a finger (dactylitis) and stippling of the nail of the same finger are particularly characteristic.
Psoriatic arthritis can show similar joint involvement as various other subgroups of juvenile idiopathic arthritis in the clinical course. In the beginning, it is often most similar to early childhood oligoarthritis. She also has severe iridocyclitis. An initial involvement of the hip joints or the involvement of a small joint (end or middle joint) should suggest the presence of psoriatic arthritis. Inflammation of the bones ( periosteum ) can also occur in the course of the disease .
Other juvenile idiopathic arthritis (ICD-10: M08.9)
Here those sub-forms of juvenile idiopathic arthritis are summarized, which meet the criteria either of none or more of the above sub-groups. Approx. 10- (20)% of the patients fall into this unclear residual group.
Among other things, a rare idiopathic juvenile osteoporosis and progressive pseudorheumatoid arthropathy are to be distinguished .
Juvenile idiopathic arthritis is a disease of unknown cause for which there is no causal therapy. On the other hand, in the majority of cases the arthritis eventually disappears. If it is possible to keep the joint, eyes or other organs away from permanent damage, the child can emerge from the disease without hindrance. The goals of the therapies are therefore to suppress pain and inflammation, avoid joint damage and ensure the child's normal development. In uveitis , the most important goal is to keep the eye working; because about 20 percent of the children affected develop a permanent visual impairment that extends to blindness .
Therapy is usually given based on the severity of the symptoms. The treatment is always carried out by a team (child, parents, pediatrician with rheumatological experience, ophthalmologist, possibly an orthodontist, physiotherapist, occupational therapist, psychologist, social worker). Treatment includes physical therapy, occupational therapy, and medication.
Physiotherapy / occupational therapy
Physiotherapy and occupational therapy should maintain and expand the range of motion of affected joints, help avoid secondary bad posture and strengthen the muscles. In the case of joint inflammation, the affected joints are held in a reflective, gentle flexion position. By positioning and relaxing the tense muscles, passive movement while gently pulling the joint, it becomes more flexible and muscles more relaxed. Then physiological movement sequences are re-established and learned. These are ultimately implemented in everyday movements. The treatment can be supplemented by cold, heat, ultrasonic and electrical applications.
The positive effect of dosed physical activity has been proven. In the acute stage, joint-relieving sports are useful. School sports are possible to a limited extent and mostly desired. An individual program should be allowed depending on the affected joints, e.g. B. renouncing jump and speed loads. Good cooperation between the school and the practitioner is helpful, with the aim of largely integrating the child into the normal social environment. What the child tolerates and where he follows his urge to move is usually not harmful.
Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)
A treatment attempt with a nonsteroidal anti-inflammatory drug such as naproxen , diclofenac , ibuprofen or indomethacin is often made at the beginning of an illness for six to ten weeks . With these drugs, pain relief occurs immediately, while the inflammatory activity is only effectively influenced after a few weeks. Side effects to the stomach and kidneys are less common in children than in adults. If this does not lead to sufficient improvement, further medication is used.
Glucocorticoids are the most effective drugs in rheumatism therapy, but have many side effects depending on the dose, type and duration of use.
A highly effective therapy option with few side effects is the injection of depot corticosteroids into an affected joint if there is oligoarthritis of large joints.
Systemically administered glucocorticoids, possibly as pulse therapy (higher-dose single doses at longer intervals), can be used as fast-acting substances in very active joint inflammation in order to reduce the time until the (slower) onset of the so-called "basic drugs" (DMARDs, "Disease Modifying Anti Rheumatic Drugs") ) to bridge. The frequency and severity of adverse effects depends on the dose and duration.
So-called basic therapeutic agents (disease modifying anti rheumatic drugs or DMARDs) and immunosuppressants are used in severe cases of juvenile idiopathic arthritis when nonsteroidal anti-inflammatory drugs or local therapeutic measures (e.g. joint injections with glucocorticoids) have not been successful. They can be combined with these drugs. All of these drugs take up to three months to take effect. The effectiveness is best documented for methotrexate , sulfasalazine and azathioprine . Methotrexate is the most common of these drugs, mostly orally. For (minor) side effects, folic acid is very often given as a supplement. The effectiveness can only be assessed after a treatment period of at least six to twelve weeks; at least six to nine months should be considered for an adequate attempt at therapy. More than 50% of children relapse after stopping it, so it should be continued long after remission. In renal insufficiency , a methotrexate therapy is not possible; this must be excluded beforehand. Serious side effects are otherwise less common in children than in adults and can often be compensated for with folic acid substitution.
The Disease Controlling Anti Rheumatic Drugs (DCARDs) from the group of biologics represent a new group of drugs. Etanercept (Enbrel ® ), a TNF inhibitor (inhibits the tumor necrosis factor ) in polyarticular juvenile chronic arthritis in children in old age, is from this group Approved for four years if methotrexate treatment was ineffective or incompatible. The humanized antibody tocilizumab specifically inhibits the interleukin-6 (IL-6) receptors, which play a central role in inflammation. In August 2011, tocilizumab was approved as the first biologic for the treatment of children aged two years and over with systemic JIA. There are also other biologics whose effectiveness has been confirmed: the TNF blockers infliximab and adalimumab , the interleukin- 1 receptor antagonist anakinra and rituximab , which acts against CD20-positive B cells.
- S2k guideline therapy of juvenile idiopathic arthritis (JIA) of the German Society for Child and Adolescent Medicine. In: AWMF online (as of 2011)
- S2k guideline for diagnosis and anti-inflammatory therapy of uveitis in juvenile idiopathic arthritis of the German Ophthalmological Society. In: AWMF online (as of 2011)
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- Still's disease: Antibodies usher in a new era of therapy