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Background: Treatment of Juvenile Idiopathic Arthritis (JIA) has improved quality of life in children and adolescents with JIA. Standardisation of care offers the chance to improve the quality of care of those patients. New studies have been published after completion of our last treatment guideline (2007). An updated consensus process is mandatory., Methods: A systematic literature analysis in PUBMED (key words: juvenile idiopathic (rheumatoid) arthritis, therapy; limits: humans, published in the last 3 years, all child 0-18 years, clinical trial) revealed 17 relevant studies. Studies relating to diagnosis of JIA, Uveitis, vaccination, transition were excluded. Representatives nominated by scientific societies and organisations were invited to consensus conferences which were hosted by a professional moderator. The following societies were invited: Berufsverband der Kinder- und Jugendärzte (BVKJ), Deutsche Gesellschaft für Kinder- und Jugendmedizin (DGKJ), Deutsche Gesellschaft für Rheumatologie (DGRh), Deutsche Ophthalmologische Gesellschaft (DOG), Deutsche Rheuma-Liga Bundesverband, Verein zur Förderung und Unterstützung rheumatologisch erkrankter Kinder und deren Eltern, Vereinigung für Kinderorthopädie, Zentraler Verband der Physiotherapeuten und Krankengymnasten (ZVK). Consensus conferences were each attended by more than 95% of the nominated representatives. Consensus statements were confirmed by nominal group technique and Delphi method., Results and Conclusion: Updated consensus statements regarding drug therapy, symptomatic and surgical management of JIA were compiled and judged strictly by the criteria of Evidence-Based Medicine (EBM)., (© Georg Thieme Verlag KG Stuttgart · New York.)

In approximately 10% of the children suffering from juvenile chronic arthritis (JCA), rheumatic iridocyclitis was also diagnosed. In 90 JCA and iridocyclitis patients we studied relative risk, clinical course and prognosis by means of several clinical and immunological parameters. Antinuclear antibodies (ANA), selective IgA-deficiency, oligoarthritis type of onset of the disease and manifestation of JCA during infancy--predominantly in girls--were quite often accompanied by a pronounced susceptibility to rheumatic iridocyclitis, while seropositivity (RF-positivity) and systemic onset of juvenile chronic arthritis normally rule out rheumatic iridocyclitis. Among HLA-B27-positive boys of school age, the acute course of rheumatic iridocyclitis, which rarely results in serious and lasting complications, is more frequent; in some cases juvenile sacroiliitis or ankylosing spondylitis are evident. The evaluation of the risk factors makes it possible to systematically define subgroups likely to develop chronic iridocyclitis and less susceptible groups of patients, thus facilitating early diagnosis and treatment as well as clinical observation of the development of chronic iridocyclitis.

Symptoms, laboratory findings and clinical course of 10 pediatric patients suffering from postinfectious arthritis are described. The postinfectious arthritis is caused by an infection with Salmonella, Versina, Brucella or Shigella. The illness results from a preceding enteritis accompanied by fever. It differs from the juvenile chronic arthritis by the more clarified etiology and pathogenesis and particularly by the markedly shorter course, the lack of functional deterioration and by normal X-ray findings of the joint structure. In the parainfectious cases a similar pathogenesis is suggested, but the time relation is not as clear; the arthritis is diagnosed ahead of the enteritis. The human leucocyte antigen HLA-B 27 can frequently (90%) be proven in patients suffering from postinfectious arthritis. This is related to a genetic disposition of this type of arthritis as it can be observed in juvenile spondylitis ankylosans and in Reiter-syndrome, which can be considered as a special type of postinfectious arthritis.

From January 1980 to June 1982, 150 children with primary onset of a joint disease during the previous 6 months were entered into a prospective non-randomized observation study. 106 of the 150 children were diagnosed as juvenile chronic arthritis cases according to the EULAR-criteria. Nine children of this group belonged to the systemic subgroup (7 girls, 2 boys). HLA-typing was performed in 100 of the 106 children: HLA-B27 was evident in the total patient group with significantly increased 41% versus 8.9% of the control group. This antigen is significantly evident in all subgroups with variously increased frequency, except in the systemic and early-onset (pauciarticular) subgroups; the frequency in the rheumatoid-factor-negative polyarticular type of juvenile chronic arthritis (JCA) was 37.9%, in the rheumatoid-factor-positive polyarticular form 33.3%, and in the oligoarthritis/sacroiliitis-group 72.2%. DR5 was significantly present in the total patient group with 32% (control group 13.4%) and in the early-onset oligoarthritis group (43.7%). The rheumatoid-factor-positive (50%) and negative polyarticular group (31%) showed a pronounced slightly significant increase.

Our studies describe symptoms, clinical course and prognosis of 60 children (out of a total of 2063 patients) suffering from chronic arthritis complicated with amyloidosis. Amyloidosis is predominantly caused by the systemic subgroup of juvenile chronic arthritis, which is characterized by high inflammation activity. The amyloidosis and the sequelae are one of the main causes of death in JCA (27 of the 90 lethal cases in our juvenile chronic arthritis unit were connected with amyloidosis). Proteinuria is the most frequent sign in the early stage of the amyloidosis. Early diagnose and treatment by cytostatic drugs like chlorambucil seems to improve the prognosis. The cumulative survival results in the existence of two different subgroup of amyloidosis.

Among 2369 children who had been admitted and treated in the Rheumatic Children's Hospital Garmisch-Partenkirchen between 1952 and 1979 under the diagnosis "juvenile chronic arthritis (JCA)" or, "collagenosis", there were 244 patients (= 10.3%) for whom in the end a different diagnosis was set. The major part of these children suffered from arthritides where there was proof of a direct or an indirect connection with bacterial infections or where there existed a corresponding suspected diagnosis (altogether 162 out of these 244 children, = 66.4%). The remaining 82 cases differentiated into 26 further diagnoses with various frequencies. Our examinations show that six criteria are found relatively frequently in arthropathies not caused by juvenile chronic arthritis. These require special consideration in the differential diagnosis: oligoarthritic onset of the disease, high fever - especially septic intermittent fever, erythema nodosum, endomyocarditis, no (or only very slight) functional resp. morphological changes of the joints.

Clinical course and prognosis of children with MCTD-Syndrome are described and six own cases are compared with the cases reported by other authors. The immunological and histological findings would seem to confirm the independence of the disease or, at least, reveal it as a particular clinical course of other connective tissue diseases. On the other hand, the initial conception, that the course of the disease can be viewed as favourable on account of the absence of kidney and CNS findings, must not be reevaluated according to our experiences of the last few years. This applies particularly to the cases of MCTD in childhood, since authors in the U.S.A. have, in the meantime, reported instances of death. One patient in our own hospital was diagnosed through biopsy as having a severe membrano-proliferative glomerulonephritis, another patient has had a severe myo-pericarditis in 1978.