[Protocols in pediatric rheumatology (PROKIND): treat-to-target in polyarticular juvenile idiopathic arthritis]. / Protokolle in der Kinderrheumatologie (PROKIND): Treat-to-Target bei polyartikulärer juveniler idiopathischer Arthritis.

BACKGROUND: The aims of the PROKIND protocols are improvement and harmonization of the diagnostics, monitoring, treatment decision and prognosis. MATERIAL AND METHODS: This article reports the results of a prospective treat-to-target observational study of patients with polyarticular juvenile idiopathic arthritis (JIA) during the first year of treatment. Disease activity was assessed with the 10-joint juvenile arthritis disease activity score (JADAS-10), functional limitation with the childhood health assessment questionnaire disability index (CHAQ-DI) and with information on overall well-being, on pain, on fatigue and on global estimation of disease activity. RESULTS: Overall, 129 patients with polyarticular JIA (rheumatoid factor, RF, positive (+) polyarthritis n = 22, RF negative (-) polyarthritis n = 133 from 23 pediatric rheumatology institutions in Germany and Austria were recruited. Patients with initial treatment with methotrexate formed cohort 1, patients with additional repeated intravenous corticosteroid pulse therapy formed cohort 2 and patients with concomitant intra-articular corticosteroid administration in at least 5 joints formed cohort 3. The mean JADAS10 showed a decrease in disease activity from 16.4 ± 6.1 to 2.8 ± 3.6 and the decrease in the CHAQ-DI from 1.0 ± 0.8 to 0.3 ± 0.5 showed the improvement in functional capacity. Similarly, improvements in quality of life, pain and fatigue were demonstrable. A JADAS inactive disease was achieved by 18.1% at month 3, 47.7% at month 6 and 66.7% at month 12. In cohort 1 a JADAS remission was achieved by 72.4%, by 50% in cohort 2 and by 69.2% in cohort 3. An escalation to treatment with biologics was necessary in 38% of patients in cohort 1, 60% in cohort 2 and 46% in cohort 3. CONCLUSION: Using a treat-to-target approach a dramatic improvement in disease activity, functional capacity and quality of life in polyarticular JIA could be achieved. Even after 12 months an inactive disease was achieved in the majority of cases.

Adalimumab versus adalimumab and methotrexate for the treatment of juvenile idiopathic arthritis: long-term data from the German BIKER registry.

OBJECTIVE: Adalimumab (ADA) has become a valuable treatment option for juvenile idiopathic arthritis (JIA). The importance of combination with methotrexate (MTX) is unclear. METHOD: Data from the German Biologics in Paediatric Rheumatology (BIKER) registry are reported. Response to treatment was analysed using JIA American College of Rheumatology (ACR) scores, 10-joint Juvenile Arthritis Disease Activity Score (JADAS10), and improvement of functional status and ACR inactive disease criteria. Compa-risons between rates of adverse events (AEs) and serious adverse events (SAEs) provided data for the safety assessment. RESULTS: Overall, 584 patients with non-systemic JIA started ADA therapy, 61% of whom received concomitant MTX treatment at baseline. The latter patients were younger (p < 0.001), with shorter disease duration (p = 0.001), more frequently had antinuclear antibodies (p = 0.04), and had higher baseline JADAS10 scores (p = 0.03). In patients with ADA monotherapy, enthesitis-related arthritis (p = 0.004) and presence of human leucocyte antigen-B27 (p = 0.008) were documented more often. Mean treatment duration in both cohorts was 15 months. Comparable last follow-up rates for JIA ACR 30/50/70/90% response, JADAS minimal disease activity, JADAS remission, and ACR inactive disease were, respectively, 75/72/64/49%, 66%, 46%, and 58% for ADA monotherapy, and 77/72/61/45%, 64%, 48%, and 55%, for ADA + MTX. During 1082 patient-years (PY) of ADA exposure, 725 AEs (67/100 PY), including 57 SAEs (5.3/100 PY), were reported. Serious infections were reported in 10 patients (0.9/100 PY) and 11 (1.0/100 PY) had varicella infections/zoster reactivation. Rates of AEs, SAEs, infectious events, and serious infections did not differ between the cohorts. Elevated transaminases (p = 0.005) and gastrointestinal events (p < 0.0001) were reported more often in the combination cohort. Two pregnancies and no deaths were reported. CONCLUSION: ADA demonstrated an acceptable risk profile and high percentages of patients in both cohorts showed sufficient treatment response. No differences in treatment response or adherence to treatment were found.

[Transition in pediatric rheumatology-experiences from the pediatric rheumatism center Sankt Augustin]. / Transition in der Kinderrheumatologie ­ Erfahrungen aus dem Kinderrheumazentrum Sankt Augustin.

BACKGROUND: Transition is a joint effort of the young patients, their families and the attending pediatric and adult rheumatologists. The quality of the transitional process for patients with rheumatic diseases was retrospectively reviewed using a standardized questionnaire. Patients were transferred to rheumatologists for adult care through personal contact, if possible. METHODS: Inclusion criteria were: diagnosis of chronic inflammatory rheumatic joint disease or systemic rheumatic disease, duration of care for >2 years in the pediatric rheumatology center and ≥2 presentations at the age of 17-18 years. A telephone interview was performed using a standardized questionnaire on the type of care, medication, diagnosis, satisfaction with the care and the transition process. RESULTS: Of the 62 enrolled patients 50 (81%) had juvenile idiopathic arthritis (JIA). In total, 40 patients (65%) were seen by an adult rheumatologist on a regular basis. Reasons for discontinuation of medical care were mainly freedom of symptoms, lack of time and non-compliance. Before and after transition, 15 and 9 patients, respectively were treated with conventional disease-modifying antirheumatic drugs (DMARD) and 27 and 27, respectively with biologics. The subjective assessment of medical care in pediatric rheumatology was (mean ± SD) 9.3 ± 1.05, in adult rheumatology 7.6 ± 1.1 and the satisfaction with the transition process was 7.7 ± 2.1. Problem issues mentioned by some patients were lack of time with the rheumatologist and long waiting periods for appointments. DISCUSSION: Individual transfer of patients from pediatric to adult rheumatology care was associated with a high degree of satisfaction in this analysis. Change of diagnosis or an unexpected termination of rheumatology treatment was not detected.

[Myositis-specific antibodies associated with juvenile dermatomyositis]. / Myositisspezifische Antikörper bei juveniler Dermatomyositis.

BACKGROUND: Juvenile dermatomyositis (JDM) is a rare autoimmune disease associated with typical skin changes and muscle weakness. Within the framework of the diagnostics, myositis-associated (MAA) and myositis-specific antibodies (MSA) can be detected. These are important for the assessment of the course of the disease and the prognosis. METHOD: In this study we searched for MAA and MSA by means of a line immunoassay in 12 currently supervised JDM patients in the Rheumatism Center Sankt Augustin. RESULTS: In 10 of the 12 patients a total of 15 myositis antibodies were detected where 3 patients each had Mi2, SRP or NXP2 antibodies, 2 had TIF-1γ antibodies and Jo1 or Mi2ß antibodies were found in 1 patient each. Of the patients two had additional PM-Scl antibodies. In the 10 patients with detected antibodies, a good phenotype-serotype correlation was found with deviation from the phenotypes described in the literature in only 3 patients. CONCLUSION: The frequent detection of certain antibodies and the good correlation with those phenotypes described in the literature, show that the determination of MSA is an important diagnostic tool to assess the course, complications and outcome and to initiate adequate therapy at an early stage.

Incidence of herpes zoster infections in juvenile idiopathic arthritis patients.

The risk of herpes zoster among patients with juvenile idiopathic arthritis (JIA) exposed to biologics has not been evaluated. We determined incidence rates of herpes zoster among children with JIA in correlation with medication at time of occurrence and total drug exposure. The German biologics register database was used to identify patients with herpes zoster. Crude infection rates and incidence ratios (IRR) were compared to published rates. Demographics and overall exposure and particular exposure time to corticosteroids, immunosuppressive drugs and biologics were analyzed. The JIA cohort included 3,042 patients with 5,557.9 person-years of follow-up; 1,628 have used corticosteroids, 2,930 methotrexate and 1,685 etanercept. In total, 17 herpes zoster events have been documented [6/1,000 patients (3.5-9.0); 3.1/1,000 patient-years (1.9-4.9)]. Thus, the incidence rate in JIA patients was higher than expected [IRR 2.9 (1.8-4.5), p < 0.001]. In all patients, the event resolved completely. There were two complications, one patient developed intercostal neuralgia, and one had a recurrent herpes zoster. Compared to the healthy population, a significant higher IRR is observed in JIA patients who received a monotherapy with etanercept or in combination with steroids and methotrexate, but not in JIA patients exposed to methotrexate without biologics. In comparison with our control group of patients treated with methotrexate, the IRR was higher for exposure to etanercept monotherapy and combination of etanercept and corticosteroids irrespective of methotrexate use. A generally higher incidence rate in JIA patients treated with etanercept was observed. No serious or refractory manifestations occurred.

Safety and efficacy of etanercept in children with juvenile idiopathic arthritis below the age of 2 years.

Etanercept is approved for the treatment of patients with juvenile idiopathic arthritis (JIA) above the age of 2 years. Experience with younger children is limited. The aim of the present study was to evaluate the efficacy and safety of treatment with etanercept in children with JIA younger than 2 years. The prospective long-term observational BIKER registry documents baseline demographics, clinical characteristics, disease activity parameters and safety issues. Efficacy was determined using the PedACR response criteria, the JADAS-10 and the proposed criteria for inactive disease and remission after 3, 6, 12, 18 and 24 months. Safety assessments were based on adverse events (AE) and serious adverse events (SAEs) reports. Between January 2001 and June 2013, a total of 13 patients including four patients with systemic JIA (sJIA), four patients with extended oligoarthritis, one patient with persistent oligoarthritis and four patients with RF negative polyarthritis were treated with etanercept. Eleven patients with follow-up assessments were analysed in our study. Prior to etanercept, all patients have been exposed to methotrexate. At last observation, 6/11 patients reached a PedACR 70 response. Two patients with sJIA and 1 with nonsystemic JIA achieved inactive disease. Tolerability was good in most of the patients. Eight AE and one SAE occurred. One patient with sJIA was affected by Hodgkin's disease 18 months after discontinuation of etanercept. New onset uveitis occurred in two patients. Reasons for discontinuation were inefficacy in three (2 sJIA), intolerance in two, remission in three (2 sJIA) and the parents' request in one patient. Etanercept seems to improve JIA patients younger than 2 years including some of the patients with sJIA. Attention should be paid to the development of malignancies and autoimmune disorders.

[Autoinflammatory syndromes in childhood]. / Autoinflammatorische Syndrome im Kindesalter.

Systemic autoinflammatory diseases are a group of hereditary and non-hereditary diseases of the innate immune system, characterized by inflammation with no apparent cause, recurrence at irregular intervals and manifestation on the skin, mucous membranes, joints, bone, gastrointestinal tract, blood vessels and the central nervous system (CNS). Amyloidosis and other possibly severe long-term complications are important. Advances in genetics and molecular biology have improved understanding of the pathogenesis of these diseases, including familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome and improved others. The vast majority of these diseases are based on activation of the interleukin-1 (IL-1) pathway, so that inhibition of IL-1 provides a therapeutic option. Other syndromes are characterized by a granulomatous inflammation. Newer autoinflammatory diseases, such as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) are, however, driven by interferons.

Intraarticular corticosteroids in refractory childhood Lyme arthritis.

Lyme arthritis caused by infection with Borrelia burgdorferi is a common late manifestation of Lyme borreliosis. Current treatment recommendations include at least one oral or intravenous antibiotic course, followed by antirheumatic therapy in case of refractory arthritis. We reviewed the course of 31 children with Lyme arthritis who had received antibiotic treatment and assessed outcome and requirement of antirheumatic therapy. Of a total of 31 patients, 23 (74%) showed complete resolution of arthritis after one or two courses of antibiotics, whereas in 8 patients (28%), steroid injections had been performed due to relapsing or remaining symptoms. All of these 8 patients showed immediate resolution of symptoms after intraarticular steroid injections. Four of them (50%) remained asymptomatic so far with a follow-up period between five up to 40 months. In two cases, multiple intraarticular corticosteroid injections were required; three patients received additional or consecutive treatment with systemic antirheumatic treatment. Patients with antibiotic refractory arthritis showed a higher rate of positivity of the IgG p58 and OspC immunoblot bands (p = 0.05) at presentation. Antibodies against OspA, an indicator of later stage infection, occurred more frequently in the refractory group without reaching significant level. No clinical marker as indicator for severe or prolonged course of Lyme arthritis was identifiable. A quarter of childhood Lyme arthritis patients were refractory to antibiotics and required antirheumatic treatment. Intraarticular steroid injections in childhood Lyme arthritis refractory to antibiotics can lead to marked clinical improvement.

Musculoskeletal pain: a new algorithm for differential diagnosis of a cardinal symptom in pediatrics.

Musculoskeletal pain (MSP) is a common childhood complaint associated with multiple differential diagnoses, including cancer. Considering the expanding spectrum of diagnostics, evaluat-ing a young patient with MSP is a challenge today, particularly for non-specialists in a primary care setting. Since childhood cancer is rare and most cardinal symptoms mimic rather non-serious diseases, misdiagnosis is not uncommon, but of significant prognostic relevance. To build the appropriate bridge between primary and secon-dary care for a child presenting with MSP, thereby preventing treatment delay and longterm sequelae, initial evaluation should follow a comprehensive, multidisciplinary, systematic and stepwise approach, which unites the patient's individual anamnestic, psychosocial, and clinical charac-teristics. After a systematic review of the literature, we generated multidisciplinarily quality-assured recommendations for efficient, rational and cost-effective primary care assessment of pediatric MSP. The algorithm promotes the identification and structured interpretation of the patient's individual clinical clues. It should serve the primary care physician to recognize when further intervention, rather than reassurance and follow-up, is needed using the minimum amount of testing to make an appropriate, prompt diagnosis in the clinical situation "child presenting with MSP". A German version of this algorithm has been published in the Guideline-Portal of The Association of the Scientific Medical Societies ("Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften", AWMF) in November 2013.

[Therapy of juvenile idiopathic arthritis in early adulthood with biologics: transition from pediatric to adult care]. / Biologikatherapie der juvenilen idiopathische Arthritis im jungen Erwachsenenalter: Übergang von der pädiatrischen in die Erwachsenenversorgung.

BACKGROUND: The majority of patients with juvenile idiopathic arthritis (JIA) need specialized care when they enter adulthood. An increasing number of these patients take biologic disease modifying antirheumatic drugs (DMARDs) at the time of transition. The biologic register BiKeR provides information about the health status and healthcare situation of JIA patients during childhood and adolescence and with their entrance into adulthood these patients are systematically transferred to JuMBO, the follow-up register for young adults with JIA treated with biologics and nonbiologic DMARDs. OBJECTIVE: The aim of this study was to investigate the healthcare situation of patients with JIA during transition from pediatric to adult care. METHODS: The current analyses included patients who were successfully transferred from the BiKeR to JuMBO registers. The DMARD treatment and patient-reported outcome (i.e. disease activity, pain and functional ability) were assessed at the last documentation in BikeR and at the first as well as the last documentation in JuMBO. RESULTS: During the transition period 1 in 10 JIA patients stopped DMARD therapy and 1 in 20 patients did not visit a physician for adults. Three-quarters of the adult JIA patients included in JuMBO (N = 811) reached adult rheumatology care. Adult rheumatologists usually continued therapy with biologics in these patients. Every second patient was still being treated with etanercept, 5 years after the start of the first treatment with biologics. Adult rheumatologists changed the biologic substance in every fourth patient, mainly because of treatment failure. In comparison to patients in regular adult rheumatology care, those who did not remain in specialized care had a higher discontinuation rate of biologics. Moreover, patients with sporadic use of medical care had a significantly poorer health status than those with a regular use of medical care at least every 6 months. CONCLUSION: The data show that there is a need for improving healthcare during the period of transition from pediatric to adult rheumatology.

[Current news from the BIKER register]. / Aktuelles aus dem BIKER-Register.

Innovative developments in the pharmacotherapy of juvenile idiopathic arthritis and especially biologics allow the formulation of new therapeutic targets, such as the rapid induction of remission with shortening of the period of active disease and therefore preventing damage and disability. These new therapies also represent a challenge to the monitoring of drug safety, the pharmacovigilance. For this purpose the Society for Paediatric and Adolescent Rheumatology has set up an early register to record achievements in treatment improvement and in addition to independently assess information on drug safety, acute tolerance and long-term safety.

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.

BACKGROUND: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries. METHODS: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients. RESULTS: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria. CONCLUSIONS: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.

The German national registry for primary immunodeficiencies (PID).

In 2009, a federally funded clinical and research consortium (PID-NET, http://www.pid-net.org) established the first national registry for primary immunodeficiencies (PID) in Germany. The registry contains clinical and genetic information on PID patients and is set up within the framework of the existing European Database for Primary Immunodeficiencies, run by the European Society for Primary Immunodeficiencies. Following the example of other national registries, a central data entry clerk has been employed to support data entry at the participating centres. Regulations for ethics approvals have presented a major challenge for participation of individual centres and have led to a delay in data entry in some cases. Data on 630 patients, entered into the European registry between 2004 and 2009, were incorporated into the national registry. From April 2009 to March 2012, the number of contributing centres increased from seven to 21 and 738 additional patients were reported, leading to a total number of 1368 patients, of whom 1232 were alive. The age distribution of living patients differs significantly by gender, with twice as many males than females among children, but 15% more women than men in the age group 30 years and older. The diagnostic delay between onset of symptoms and diagnosis has decreased for some PID over the past 20 years, but remains particularly high at a median of 4 years in common variable immunodeficiency (CVID), the most prevalent PID.

Genotype-phenotype and genotype-origin correlations in children with mediterranean fever in Germany - an AID-net study.

Familial Mediterranean fever (FMF) is the most inherited common autoinflammatory disease (AID) with mutations in the MEFV (MEditerraneanFeVer) gene.The Mor- and Pras-Score modified for children and C-reactive protein (CRP) were used to assess FMF disease severity in Germany. We evaluate the applicability of the 2 severity scores and the correlations between ethnic origin, phenotype, and genotype.Among 242 children (median 5 age at diagnosis), we detected 431 pyrin mutations and 22 different sequence variants, including one new mutation (p.Gly488Asp). The 5 most -frequent alterations were p.Met694Val (55.2%), p.Met680lle (11.8%), p.Val726Ala (10%), p.Glu148Gln (7.9%) and p.Met694IIe (2.3%). The prevailing ancestries of 223 cases were Turkish (82.5%) and Lebanese (8.1%). Homozygous p.Met694Val substitution (30.2%) was associated with a more severe disease activity by Mor-Score, as well as with a higher mean CRP (74 mg/l) compared to patients with other mutations. Indeed, Mor- and Pras-Score were inconsistent with each other. A typical distribution of mutations in different ethnic populations was obvious, but not statistically verifiable due to the low number of cases.The homozygous p.Met694Val substitution was associated with a more severe disease activity in our German cohort. The common severity scores were inconsistent in -children.

[Biologics register JuMBO. Long-term safety of biologic therapy of juvenile idiopathic arthritis]. / Biologikaregister JuMBO. Langzeitsicherheit von Biologikatherapie bei juveniler idiopathischer Arthritis.

In recent years the treatment of juvenile idiopathic arthritis (JIA) has changed dramatically. Nowadays one out of three children with polyarticular JIA is treated with a biologic drug; however, knowledge about the long-term safety of biologics is still limited. Information on drug safety is collected in the JIA biologic register (BiKeR) and the follow-up register juvenile arthritis methotrexate/biologics long-term observation (JuMBO). The latter currently includes information on more than 700 young adults most of whom were treated with etanercept and prospectively followed for more than 5 years. Preliminary data on the long-term safety of etanercept for JIA are therefore available. Over an observation period of 1,800 etanercept exposure-years, events of particular interest, such as malignancies, serious infections and new onset immune-mediated diseases have been recorded which occurred at rates of 0.1, 1.1 and 0.9/100 patient-years, respectively. Overall, new safety risks were not detected during long-term etanercept exposure. Moreover, JuMBO has also provided information on the long-term outcome of JIA and initial evidence suggests that JIA outcome, especially in functional aspects has improved in the biologic era. Data from BiKeR and JuMBO contribute to the risk-benefit assessment of biologic drugs which have been implemented in the routine treatment of JIA.

Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis.

Uveitis in juvenile idiopathic arthritis (JIA) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first-choice therapy, and immunosuppression is commonly used. However, treatment has not been standardized. Representatives from the German Ophthalmological Society, Society for Childhood and Adolescent Rheumatology, and the German Society for Rheumatology reached consensus on a standardized treatment strategy according to disease severity in the individual patient. The recommendations were based on a systematic literature analysis in MEDLINE and consensus expert meetings. Evidence and recommendations were graded, and an algorithm for anti-inflammatory treatment and final statements confirmed in a Delphi method. An interdisciplinary, evidence-based treatment guideline for JIA uveitis is presented.

Results of the German ESPED-recording of new patients with juvenile dermatomyositis (JDM).

In a nation-wide registration project 38 incident cases of juvenile dermatomyositis were collected in Germany over a 2-year-period. Diagnostic methods as well as the primary treatment for these patients were recorded. Detailed information was available for 25 of these patients. Diagnostic as well as therapeutic decisions varied widely. Steroids were used in almost all of the 25 patients either as oral or as parenteral pulse therapy, additional immunosuppressive drugs were used in 52%. We plan to establish national consensus recommendations for diagnostic and therapeutic standards in JDM. Due to the rarity of JDM clinical trials will have to be performed on an international basis.

[Evidence and consensus based treatment guidelines 2010 for juvenile idiopathic arthritis by the German Society of Paediatric Rheumatology]. / Interdisziplinäre S2-Therapieleitlinie der Juvenilen Idiopathischen Arthritis (2. Auflage).

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).