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Abbildung 3: Ärztliche Behandlungsbeteiligung bei gesetzlich krankenversicherten Kindern und Jugendlichen im Altersbereich 0 bis 19 Jahre mit prävalenter bzw. inzidenter Erkrankung an juveniler idiopathischer Arthritis (JIA) nach erkrankungsrelevanten Facharztgruppen einschließlich der Zusatzbezeichnung Kinderrheumatologie im Jahr 2015; angegeben sind die Versorgungsanteile der Fachgruppen an allen JIA-Fällen in Prozent (%). Anmerkung: An der individuellen Versorgung von JIA-Patienten können pro Kalenderjahr mehrere Ärzte und damit auch Fachgruppen beteiligt sein, sodass die Summe der prozentualen Anteile aller Facharztgruppen einen Wert über 100 % ergibt.
Source publication
Report in German
Abstract
Hintergrund: Die juvenile idiopathische Arthritis (JIA) ist eine chronische, autoimmune Systemerkrankung, die durch eine mindestens über sechs Wochen bestehende Gelenkentzündung unklarer Genese bei Kindern und Jugendlichen unter 16 Jahren charakterisiert ist. Belastbare Daten zur Prävalenz und Inzidenz in Deutschland exist...
Citations
... The research databases used for the analyses are representative of the German SHI population in terms of age and sex [23]; however, regional representation is not guaranteed. The two databases we used may have different regional representations, and region has been observed in other studies to play a role in JIA prevalence rates [38] and even JIA treatment habits [39]. Additionally, with low case numbers attributable to the rarity of the disease, the patient pool from which we draw any conclusions is limiting. ...
(1) Background: Achieving inactive disease decreases long-term joint damage in patients with polyarticular juvenile idiopathic arthritis (polyJIA). The aim of our study was to describe average time to treatment and medication changes over time. (2) Methods: Incident polyJIA patients were retrospectively identified in the InGef and WIG2 longitudinal health claims databases. Drug escalation level changes were evaluated longitudinally and cross-sectionally across three years, as follows: no treatment, glucocorticoids (GCs) and/or non-steroidal anti-inflammatory drugs (NSAIDs), conventional synthetic disease-modifying antirheumatic drugs (csDMARDs), and biological disease-modifying antirheumatic drugs (bDMARDs). (3) Results: On average, newly diagnosed polyJIA patients received their first csDMARD prescription after 128 days and their first bDMARD prescription after 327 days. More patients were treated with csDMARDs than with bDMARDs at diagnosis; however, 24% and 12% (InGef and WIG2 databases, respectively) had no JIA treatment. After three years, 45% and 31% were not taking any treatments, while 18% and 36% were prescribed bDMARDs. Among patients initiating bDMARDs, most continued treatment for three years, with some switching to csDMARDs or discontinuing treatment. Patients treated only with csDMARDs took them longer, compared to those additionally taking other DMARDs. Patients treated with bDMARDs took them about twice as long as the csDMARDs they took prior. (4) Conclusion: A substantial number of patients with polyJIA are not treated as intensively as guidelines recommend.
... 23,3 % (RF-pJIA) verantwortlich (Quelle: Einrichtungsauswertung Kerndokumentation 2021, DRFZ). Auf Basis der epidemiologischen Daten zur JIA in Deutschland [6] errechnen sich jährliche Inzidenzen von 0,38/100 000 (RF+ pJIA), 3,8/100 000 (RF-pJIA) bzw. Prävalenzen von 2,3/100 000 (RF+ pJIA) und 23/100 000 (RF-pJIA). ...
Juvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of myositis-specific autoantibodies (MSA). In contrast to dermatomyositis in adults, in children and adolescents there are no associations with malignant diseases. The variable expression, the rarity of the disease and the risk of long-term damage and complications necessitate pediatric rheumatological experience in the diagnostics and treatment. Recently, new approaches in drug treatment have substantially improved the outcome and prognosis but a multidisciplinary treatment (including physicians, physiotherapists, psychologists, social workers) is mandatory, especially in the first phases of the disease. Particularly important is a professionally correct treatment of the functional sequelae, which are a particular focus of this article.
... Database studies offer a widespread analysis of the population, however, they rely heavily on case definitions and sample data size and representativeness. To help fill this knowledge gap, we used a more broad range of diagnosis codes than similar study designs [17] thereby improving sensitivity, however defining timing of codes so as to maintain specificity. Applying these criteria to two large health claims databases (covering over 8 million SHI German residents in total), we evaluated prevalence and incidence of JIA, and frequently associated comorbidities of JIA and polyJIA in Germany. ...
... Since children or adolescents occasionally receive adult diagnosis codes [17], we included adult codes in our JIA criteria (in those aged 2-15). ...
... We found overall 2018 JIA prevalence rates of 133.21 and 167.76, and incidence rates of 34.17 and 59.99 per 100,000 German population in the WIG2 and InGef databases, respectively (Fig. 1). These rates are higher than those from another study using SHI claims data, with prevalence rates of 73.4 to 101.5 cases per 100,000 between 2009 and 2015, and incidence rates 16.0 and 17.4 per 100,000 from 2011 to 2015 [17]. The main reason is likely the use of different case definitions; this study excluded inpatient diagnoses and some ICD-10 codes typically used for adults, both of which we included in our analysis (for example codes M05, M06 and M07). ...
Background
Juvenile idiopathic arthritis (JIA) describes heterogenous categories of chronic inflammatory rheumatic conditions of unknown origin in children and adolescents. Epidemiological data in the literature vary, depending on geographic location, ethnicity and the case definition used. We evaluated epidemiology, especially that of the categories defined by the International League of Associations for Rheumatology (ILAR).
Methods
Using data from two different longitudinal health claims databases (WIG2 and InGef) from January 1st, 2013 to December 31st, 2019, we looked at patients aged 2 to 15 years old with at least one main inpatient or two secondary inpatient/verified outpatient ICD-10 diagnoses in at least two different quarters within one calendar year. We calculated prevalence and incidence (per 100,000 patients) and extrapolated data to the entire German population, looking at differences in gender and age groups. Additionally, we collected data on “other” not necessary comorbidities in our JIA patient population.
Results
Of the 3–4 million patients in the databases (respectively) in 2018, we found a total of 546 (WIG2) and 849 (InGef) patients that met our JIA case definition, with an incidence of 34 (29–41) and 60 (53–67) and prevalence of 133 (122–145) and 168 (157–179). Both incidence and prevalence throughout the age range were mostly higher in females than males, however the difference between females and males increased with increasing age. Of the ILAR categories, oligoarthritis was the most prevalent (70 and 91 per 100,000), with about half of our JIA patients in this category, followed by undifferentiated arthritis (49 and 56 cases per 100,000) and rheumatoid factor negative (RF-) (31 and 39 per 100,000). Incidence in 2018 was the highest in these three categories. Atopic dermatitis, vasomotor and allergic rhinitis, and uveitis were the pre-defined comorbidities seen most often in both databases.
Conclusion
This study provides current incidence and prevalence JIA data in Germany, contributing to knowledge on burden of disease and tools for healthcare planning.
... One possible explanation could be that females are more affected by internalizing disorders during adolescence [9]. Furthermore, more females are affected by JIA [81]. In addition, male adolescents with emotional burdens tend to show less help-seeking behavior than female adolescents [82,83]. ...
Background
Adolescents and young adults (AYA) with a chronic medical condition show an increased risk for developing mental comorbidities compared to their healthy peers. Internet- and mobile-based cognitive behavioral therapy (iCBT) might be a low-threshold treatment to support affected AYA. In this randomized controlled pilot trial, the feasibility and potential efficacy of youthCOACHCD, an iCBT targeting symptoms of anxiety and depression in AYA with chronic medical conditions, was evaluated.
Methods
A total of 30 AYA (Mage 16.13; SD= 2.34; 73% female), aged 12-21 years either suffering from cystic fibrosis, juvenile idiopathic arthritis or type 1 diabetes, were randomly assigned to either a guided version of the iCBT youthCOACHCD (IG, n=15) or to a waitlist control group (CG, n=15), receiving an unguided version of the iCBT six months post-randomization. Participants of the IG and the CG were assessed before (t0), twelve weeks after (t1) and six months after (t2) randomization. Primary outcome was the feasibility of the iCBT. Different parameters of feasibility e.g. acceptance, client satisfaction or potential side effects were evaluated. First indications of the possible efficacy with regard to the primary efficacy outcome, the Patient Health Questionnaire Anxiety and Depression Scale, and further outcome variables were evaluated using linear regression models, adjusting for baseline values.
Results
Regarding feasibility, intervention completion was 60%; intervention satisfaction (M = 25.42, SD = 5.85) and perceived therapeutic alliance (M = 2.83, SD = 1.25) were moderate and comparable to other iCBTs. No patterns emerged regarding subjective and objective negative side effects due to participation in youthCOACHCD. Estimates of potential efficacy showed between group differences, with a potential medium-term benefit of youthCOACHCD (β = -0.55, 95%CI: -1.17; 0.07), but probably not short-term (β = 0.20, 95%CI: -0.47; 0.88).
Conclusions
Our results point to the feasibility of youthCOACHCD and the implementation of a future definitive randomized controlled trial addressing its effectiveness and cost-effectiveness. Due to the small sample size, conclusions are premature, however, further strategies to foster treatment adherence should be considered.
Trial registration
The trial was registered at the WHO International Clinical Trials Registry Platform via the German Clinical Trials Register (ID: DRKS00016714, 25/03/2019).
... B ronchial asthma (prevalence about 4%), psoriasis (about 0.7%), chronic inflammatory bowel disease (0.1%), and juvenile idiopathic arthritis (about 0.1%) are among the most common chronic inflammatory diseases in children. They are immune-modulated diseases (1)(2)(3). An important part in their pathogenesis is played by cytokines, including interleukin (IL)-1, IL-6 in juvenile idiopathic arthritis with systemic onset [sJIA], and tumor necrosis factor-α (TNFα) in polyarticular forms of juvenile idiopathic arthritis, psoriasis, and chronic inflammatory bowel disease) ( Figure 1). ...
... (11) Busse (12) Lanier (13 Psoriasis Area and Severity Index, which combines the assessment of the severity of lesions and the area affected into a single score in the range 0 (no disease) to 72 (maximal disease). The body is divided into four sections for assessment of the extent of involvement (0 to 100%) and each section scored for erythema, induration, and desquamation (grades [1][2][3][4]. PASI 75 gives the percentage of patients whose PASI score went down by 75% or more from baseline. PGA, Physician Global Assessment * 7 Refractory to first-line therapy (e.g., intra-articular and/or systemic steroids, steroid-based eye drops, nonsteroidal antirheumatic drugs, methotrexate/disease-modifying antirheumatic drug (DMARD), or other biologic agents) * 8 JIA ACR 30 (or 50, 70, 75): Improvement by 30% (or 50%, 70%, 75%) or more in three or more of the six variables of the ACR core outcome variables for JIA: (1) PGA (10-cm visual analog scale), (2) parent/patient assessment of overall wellbeing (10-cm visual analog scale), (3) functional ability, (4) number of joints with active arthritis, (5) number of joints with limited range of movement, and (6) ESR, with no more than one variable that has worsened by more than 30%. ...
Background:
Monoclonal antibodies (mAb) and fusion proteins (FP) are increasingly being used in children and adolescents. In this review, we analyze the evidence for their safety and efficacy in the treatment of the most common chronic inflammatory diseases.
Methods:
We systematically searched PubMed, AWMF.org, and other databases for high-quality trials (i.e., randomized controlled trials with clinical primary endpoints) and guidelines published at any time up to 10 December 2018 that dealt with mAb and FP that are approved for pediatric use. The search term was "monoclonal anti- body/fusion protein [e. g. adalimumab] AND children."
Results:
The 620 hits included 25 high-quality trials (20 of them manufacturer- sponsored) on 9 mAb/FP (omalizumab, adalimumab, etanercept, ustekinumab, infliximab, golimumab, anakinra, canakinumab, tocilizumab, and abatacept), as well as 6 guidelines (3 each of levels S3 and S2k) on the treatment of bronchial asthma, psoriasis, juvenile idopathic arthritis, and chronic inflammatory bowel diseases. For none of these conditions are mAb and FP the drugs of first choice. Adverse drug effects are rare but sometimes severe (infection, immune dysregulation, tumors).
Conclusion:
The retrieved trials have deficiencies that make it difficult to reliably evaluate the efficacy, safety, and utility of mAb/FP for children and adolescents with chronic inflammatory diseases. mAb/FP nonetheless represent a treatment option to be considered in case conventional immune-modulating drugs are ineffective. Researcher-initiated, high-quality trials and manufacturer-independent, systematic long-term evaluations of adverse effects (e.g., tumors) are sorely needed.
Zusammenfassung
Zielsetzung
Es erfolgt eine aktualisierte Schätzung der Prävalenz entzündlich rheumatischer Erkrankungen (ERE) in Deutschland.
Methodik
Mit einer systematischen Literaturrecherche in PubMed und Web of Science (letzte Suche am 08.11.2022) wurden Originalartikel (regionale und bundesweite Surveys und Routinedatenanalysen für Arthritiden, Kollagenosen und Vaskulitiden) zur Prävalenz von ERE für den Zeitraum 2014 bis 2022 identifiziert. Datenquellen, Erhebungszeitraum, Diagnosedefinition und das Risiko einer Verzerrung werden berichtet. Die Prävalenzen wurden anhand der verfügbaren Daten unter Berücksichtigung internationaler Angaben geschätzt.
Ergebnisse
Die Suche durch 2 Autorinnen ergab 263 Treffer, von denen 18 Routinedatenanalysen und 2 Surveys die Einschlusskriterien erfüllten. Die Prävalenzangaben lagen bei 0,42–1,85 % (rheumatoide Arthritis), 0,32–0,5 % (ankylosierende Spondylitis), 0,11–0,32 % (Psoriasisarthritis), 0,037–0,14 % (systemischer Lupus erythematodes), 0,07–0,77 % (Sjögren/Sicca-Syndrom), 0,14–0,15 % (Polymyalgia rheumatica, ab 40 Jahre), 0,04–0,05 % (Riesenzellarteriitis, ab 50 Jahre) und 0,015–0,026 % (ANCA-assoziierte Vaskulitis). Das Bias-Risiko war in 13 Studien moderat, in 7 Studien hoch. Anhand dieser Ergebnisse schätzen wir die Prävalenz von ERE in Deutschland auf 2,2–3,0 %. Dies entspricht in etwa 1,5 bis 2,1 Mio. Betroffenen. Die Prävalenz der juvenilen idiopathischen Arthritis wurde mit ca. 0,10 % (0,07–0,13 %) der 0‑ bis 18-Jährigen angegeben, was etwa 14.000 Kindern und Jugendlichen in Deutschland entspricht.
Schlussfolgerung
Dieses systematische Review zeigt einen Anstieg der Prävalenzen von ERE in Deutschland, basiert jedoch fast ausschließlich auf Routinedatenanalysen. In Ermangelung mehrstufiger Bevölkerungsstudien sind die vorliegenden Daten bei moderat bis hohem Verzerrungsrisiko insgesamt unsichere Quellen für Prävalenzschätzungen.
Zusammenfassung
Im Gebiet der Kinderrheumatologie gab es in den letzten Jahrzehnten immense Fortschritte, die sowohl die Diagnostik, als auch die Therapie nachhaltig verbessert haben. Obwohl erst seit 2003 in Deutschland offiziell als Zusatzbezeichnung anerkannt, stehen heutzutage über 200 Kinder- und Jugendrheumatologen (d. h. 1,4 Kinderrheumatologen pro 100 000 Kinder) für die Erkennung und Behandlung von rheumatischen Erkrankungen bei Kindern und Jugendlichen bundesweit zur Verfügung. Neue Erkenntnisse in der Pathogenese rheumatischer Erkrankungen und die sich stetig weiterentwickelnde genetische Diagnostik haben das rheumatische Krankheitsspektrum und die Behandlungsmöglichkeiten dramatisch erweitert Internationale Forschungsnetzwerke und eine spezielle Gesetzgebung für die Entwicklung von pädiatrischen Medikamenten führten zur Zulassung von zahlreichen neuen Rheumamedikamenten, deren Sicherheit im klinischen Alltag seit der Jahrtausendwende systematisch in Deutschland untersucht wird. Maßnahmen zur Sicherung der Versorgungsqualität wurden implementiert, Standardinstrumente zur Bewertung der Krankheitsaktivität und Krankheitslast aus Patientensicht eingeführt sowie Initiativen zur Verbesserung der Versorgung Betroffener (z. B. die ProKind-Initiative) auf den Weg gebracht. Diese Veränderungen haben die Prognose und Lebensperspektive rheumakranker Kinder und Jugendlicher verbessert, wenngleich noch weiterer Optimierungsbedarf besteht.
The treatment of juvenile idiopathic arthritis (JIA) has made substantial progress within the last 25 years. Modern medicinal treatment enables inflammatory activity of the disease to be controlled in most of the cases. Mutilating courses of disease, which were formerly the rule have now become the exception. Today remission of disease is the aim of pediatric rheumatological treatment. Apart from effective control of inflammation this includes complete restoration of functional abilities of affected joints and the surrounding structures also affected. To achieve this goal a holistic and foresighted view of each patient’s course is required. Therefore, even in an apparently uncomplicated course of disease in some cases of JIA it is advisable to plan an early interdisciplinary consultation including the pediatric rheumatologist and the orthopedic surgeon, in order to discuss an early surgical intervention, which can then be carried out in a timely manner, if necessary. This article provides an overview of the orthopedic rheumatological indications and options.
