Verlauf der Juvenilen Idiopathischen Arthritis im Erwachsenenalter

Hintergrund: Ein großer Teil der JIA-Patienten leidet im Verlauf des Lebens unter chronischen oder wiederkehrenden Schmerzen und muss aufgrund der Erkrankung mit Einschränkungen im Alltag leben. Das Outcome der JIA konnte im Laufe der letzten zwei Jahrzehnte durch neue Therapien erheblich verbessert werden. Der größte Fortschritt wurde durch die Einführung von Biologika erreicht, die allerdings im Verdacht stehen das Risiko für das Auftreten von Malignomen zu erhöhen. Zielsetzung: Das Ziel dieser Arbeit war es, das Outcome von JIA-Patienten im Hinblick auf die Lebensqualität und die Inzidenz von Malignomen zu untersuchen. Methoden: Patienten, die zwischen 1952 und 2010 im Deutschen Zentrum für Kinder- und Jugendrheumatologie (DZKJR) wegen einer rheumatischen Erkrankung in Behandlung waren (N=10.580), erhielten Anfang 2012 einen Fragebogen per Post. Erfragt wurden soziodemografische Faktoren, Details zur Rheumaerkrankung, zur Lebensqualität (EQ5D-Fragebogen) und bisherigen Krebsdiagnosen. Für alle Studienteilnehmer wurden die Eigenangaben zur JIA Diagnose mit Hilfe der Krankenakten überprüft und nur solche Patienten eingeschlossen, deren Diagnose durch die Akte bestätigt werden konnte. Von diesen wurden diejenigen, die über eine bösartige Krebsdiagnose berichteten, als Fall definiert und mit bis zu vier Kontrollen aus der gleichen Kohorte nach Geschlecht, Alter (+/- 2 Jahre) und dem Datum der ersten Aufnahme im DZKJR (+/- 2 Jahre) gematcht. Im Frühjahr 2013 erhielten alle Fälle und Kontrollen einen zweiten Fragebogen, mit dem sie zur Therapie seit JIA-Diagnosestellung befragt wurden (genestete Fall-Kontroll-Studie). Neben deskriptiver Statistik wurden multivariate logistische Regressionsmodelle erstellt. Um die Krebsinzidenz in Relation zur Allgemeinbevölkerung zu betrachten, wurden standardisierte Inzidenzraten (SIR) und die dazugehörigen 95%-Konfidenzintervalle (95%-KI) berechnet. Ergebnisse: Insgesamt beantworteten 6127 Patienten den ersten Fragebogen (Response 66%), 3698 von ihnen hatten eine JIA. Die JIA-Patienten waren zwischen 3 und 73 Jahre alt, 64% waren weiblich. Ihre Lebensqualität war im Vergleich zur Allgemeinbevölkerung in allen Bereichen statistisch signifikant eingeschränkt, vor allem im Bereich Schmerzen. Das weibliche Geschlecht, höheres Alter, niedrigere Bildung, sich aktuell in rheumatischer Behandlung zu befinden und das Vorliegen einer Behinderung waren mit niedrigerer Lebensqualität assoziiert. Bei 48 Patienten wurde ein Malignom diagnostiziert, 35 davon traten bei Frauen auf. Die häufigsten Krebsarten waren Melanom (n=11), Zervixkarzinom (n=8) und Mammakarzinom (n=7). Insgesamt war die Inzidenz von Krebs im Vergleich zur Allgemeinbevölkerung nicht erhöht (SIR 0,99 (95%-KI: 0,69; 1,29)), bei weiblichen JIA-Patienten wurde häufiger ein Melanom beobachtet als bei Frauen aus der Allgemeinbevölkerung (SIR: 3.21 (95%-KI: 1.60; 5.73). In die genestete Fall-Kontroll-Studie wurden 37 Fälle und 125 Kontrollen eingeschlossen (Response 92%). Es wurden keine statistisch signifikanten Unterschiede zwischen Tumorpatienten und Kontrollen hinsichtlich ihrer Medikamenteneinnahme gefunden. Schlussfolgerung: Im Vergleich zur Allgemeinbevölkerung waren die JIA-Patienten in ihrer Lebensqualität benachteiligt, ihr Risiko an einem Malignom zu erkranken war unabhängig von der Medikamenteneinnahme nicht erhöht. Lediglich bei den Frauen fand sich ein leicht erhöhtes Risiko für das Auftreten eines Melanoms.Background: A substantially proportion of all JIA patients suffer from chronic or recurrent pain and have to live with limitations in daily life. Outcome of JIA has been considerably improved by new forms of therapy over the past two decades. The greatest progress has been achieved by the introduction of biologicals, which, however, are suspected to increase the risk of the occurrence of malignancies. Aim: The objective of this research was to investigate the outcome of JIA patients with regard to the health-related quality of life (HRQOL) and the incidence of malignancy. Methods: Patients who were under medical treatment in the German Centre for Pediatric and Adolescent Rheumatology (GCPAR) (Garmisch-Partenkirchen, Germany) between 1952 and 2010 received a self-administered standardized questionnaire by mail at the beginning of 2012 (n=10,580). The questionnaire included sociodemographic factors, details of the rheumatic disease, questions about their HRQOL (EQ5D questionnaire) and previous cancer diagnoses. For all study participants the self-reported JIA diagnosis was verified by means of the medical records and only those patients whose diagnosis was confirmed with the medical documents were included. Among these, the ones that reported a malignant tumor were defined as cases and were individually matched by sex, age (+/- 2 years) and date of first admission to the hospital (+/- 2 years) with up to four controls each. In the spring of 2013, all cases and controls received a second self-administered standardized questionnaire to raise drug intake (nested case-control-study). In addition to descriptive statistics, multivariate logistic regression models were calculated. To compare cancer incidence in the JIA cohort with the German general population, standardized incidence ratios (SIRs) and their corresponding 95% confidence intervals (95%-CIs) were calculated. Results: Overall, 6127 patients responded to the first questionnaire (response 66%), 3698 of them had a JIA. Age of study participants ranged from 3 to 73 years, 64% were female. HRQOL of JIA patients was significantly lower in all domains compared to the general population, especially in the area of pain. Female sex, older age, lower education, currently being in rheumatic treatment and the existence of a disability were associated with lower HRQOL. A malignancy was diagnosed in 48 patients, 35 of them occurred in women (74%). The most frequently observed type of cancer were melanoma (n=11), cervical cancer (n=8) and breast cancer (n=7). Compared with the general population the overall incidence of cancer was not increased (SIR: 0.99 (95% CI: 0.69; 1.29)); in female JIA patients melanoma was more frequently observed than in women from the general population (SIR: 3.21 (95% CI: 1.60; 5.73)). In the nested case-control study 37 cases and 125 controls were included (response 92%). There were no significant differences between cases and controls regarding their medication. Conclusion: Compared to the general population JIA patients were disadvantaged in their HRQOL; their risk of malignancy was not increased regardless of their medication. But for woman a slightly increased risk for melanoma was found

Verlauf der Juvenilen Idiopathischen Arthritis im Erwachsenenalter

Hintergrund: Ein großer Teil der JIA-Patienten leidet im Verlauf des Lebens unter chronischen oder wiederkehrenden Schmerzen und muss aufgrund der Erkrankung mit Einschränkungen im Alltag leben. Das Outcome der JIA konnte im Laufe der letzten zwei Jahrzehnte durch neue Therapien erheblich verbessert werden. Der größte Fortschritt wurde durch die Einführung von Biologika erreicht, die allerdings im Verdacht stehen das Risiko für das Auftreten von Malignomen zu erhöhen. Zielsetzung: Das Ziel dieser Arbeit war es, das Outcome von JIA-Patienten im Hinblick auf die Lebensqualität und die Inzidenz von Malignomen zu untersuchen. Methoden: Patienten, die zwischen 1952 und 2010 im Deutschen Zentrum für Kinder- und Jugendrheumatologie (DZKJR) wegen einer rheumatischen Erkrankung in Behandlung waren (N=10.580), erhielten Anfang 2012 einen Fragebogen per Post. Erfragt wurden soziodemografische Faktoren, Details zur Rheumaerkrankung, zur Lebensqualität (EQ5D-Fragebogen) und bisherigen Krebsdiagnosen. Für alle Studienteilnehmer wurden die Eigenangaben zur JIA Diagnose mit Hilfe der Krankenakten überprüft und nur solche Patienten eingeschlossen, deren Diagnose durch die Akte bestätigt werden konnte. Von diesen wurden diejenigen, die über eine bösartige Krebsdiagnose berichteten, als Fall definiert und mit bis zu vier Kontrollen aus der gleichen Kohorte nach Geschlecht, Alter (+/- 2 Jahre) und dem Datum der ersten Aufnahme im DZKJR (+/- 2 Jahre) gematcht. Im Frühjahr 2013 erhielten alle Fälle und Kontrollen einen zweiten Fragebogen, mit dem sie zur Therapie seit JIA-Diagnosestellung befragt wurden (genestete Fall-Kontroll-Studie). Neben deskriptiver Statistik wurden multivariate logistische Regressionsmodelle erstellt. Um die Krebsinzidenz in Relation zur Allgemeinbevölkerung zu betrachten, wurden standardisierte Inzidenzraten (SIR) und die dazugehörigen 95%-Konfidenzintervalle (95%-KI) berechnet. Ergebnisse: Insgesamt beantworteten 6127 Patienten den ersten Fragebogen (Response 66%), 3698 von ihnen hatten eine JIA. Die JIA-Patienten waren zwischen 3 und 73 Jahre alt, 64% waren weiblich. Ihre Lebensqualität war im Vergleich zur Allgemeinbevölkerung in allen Bereichen statistisch signifikant eingeschränkt, vor allem im Bereich Schmerzen. Das weibliche Geschlecht, höheres Alter, niedrigere Bildung, sich aktuell in rheumatischer Behandlung zu befinden und das Vorliegen einer Behinderung waren mit niedrigerer Lebensqualität assoziiert. Bei 48 Patienten wurde ein Malignom diagnostiziert, 35 davon traten bei Frauen auf. Die häufigsten Krebsarten waren Melanom (n=11), Zervixkarzinom (n=8) und Mammakarzinom (n=7). Insgesamt war die Inzidenz von Krebs im Vergleich zur Allgemeinbevölkerung nicht erhöht (SIR 0,99 (95%-KI: 0,69; 1,29)), bei weiblichen JIA-Patienten wurde häufiger ein Melanom beobachtet als bei Frauen aus der Allgemeinbevölkerung (SIR: 3.21 (95%-KI: 1.60; 5.73). In die genestete Fall-Kontroll-Studie wurden 37 Fälle und 125 Kontrollen eingeschlossen (Response 92%). Es wurden keine statistisch signifikanten Unterschiede zwischen Tumorpatienten und Kontrollen hinsichtlich ihrer Medikamenteneinnahme gefunden. Schlussfolgerung: Im Vergleich zur Allgemeinbevölkerung waren die JIA-Patienten in ihrer Lebensqualität benachteiligt, ihr Risiko an einem Malignom zu erkranken war unabhängig von der Medikamenteneinnahme nicht erhöht. Lediglich bei den Frauen fand sich ein leicht erhöhtes Risiko für das Auftreten eines Melanoms.Background: A substantially proportion of all JIA patients suffer from chronic or recurrent pain and have to live with limitations in daily life. Outcome of JIA has been considerably improved by new forms of therapy over the past two decades. The greatest progress has been achieved by the introduction of biologicals, which, however, are suspected to increase the risk of the occurrence of malignancies. Aim: The objective of this research was to investigate the outcome of JIA patients with regard to the health-related quality of life (HRQOL) and the incidence of malignancy. Methods: Patients who were under medical treatment in the German Centre for Pediatric and Adolescent Rheumatology (GCPAR) (Garmisch-Partenkirchen, Germany) between 1952 and 2010 received a self-administered standardized questionnaire by mail at the beginning of 2012 (n=10,580). The questionnaire included sociodemographic factors, details of the rheumatic disease, questions about their HRQOL (EQ5D questionnaire) and previous cancer diagnoses. For all study participants the self-reported JIA diagnosis was verified by means of the medical records and only those patients whose diagnosis was confirmed with the medical documents were included. Among these, the ones that reported a malignant tumor were defined as cases and were individually matched by sex, age (+/- 2 years) and date of first admission to the hospital (+/- 2 years) with up to four controls each. In the spring of 2013, all cases and controls received a second self-administered standardized questionnaire to raise drug intake (nested case-control-study). In addition to descriptive statistics, multivariate logistic regression models were calculated. To compare cancer incidence in the JIA cohort with the German general population, standardized incidence ratios (SIRs) and their corresponding 95% confidence intervals (95%-CIs) were calculated. Results: Overall, 6127 patients responded to the first questionnaire (response 66%), 3698 of them had a JIA. Age of study participants ranged from 3 to 73 years, 64% were female. HRQOL of JIA patients was significantly lower in all domains compared to the general population, especially in the area of pain. Female sex, older age, lower education, currently being in rheumatic treatment and the existence of a disability were associated with lower HRQOL. A malignancy was diagnosed in 48 patients, 35 of them occurred in women (74%). The most frequently observed type of cancer were melanoma (n=11), cervical cancer (n=8) and breast cancer (n=7). Compared with the general population the overall incidence of cancer was not increased (SIR: 0.99 (95% CI: 0.69; 1.29)); in female JIA patients melanoma was more frequently observed than in women from the general population (SIR: 3.21 (95% CI: 1.60; 5.73)). In the nested case-control study 37 cases and 125 controls were included (response 92%). There were no significant differences between cases and controls regarding their medication. Conclusion: Compared to the general population JIA patients were disadvantaged in their HRQOL; their risk of malignancy was not increased regardless of their medication. But for woman a slightly increased risk for melanoma was found

Association between drug intake and incidence of malignancies in patients with Juvenile Idiopathic Arthritis: a nested case-control study

Background: Several medications for treatment of Juvenile Idiopathic Arthritis (JIA) are considered to be carcinogenic. Therefore, the aim was to assess whether there is an association between therapeutic interventions and malignancies in JIA patients. Findings: A nested case-control study was carried out within a retrospective cohort study of 3698 JIA patients diagnosed between 1952 and 2010. All 48 JIA patients with a diagnosis of a malignant tumour and up to four matched controls for each received a questionnaire about their use of medication. Subsequently treatment was compared between cases and controls and analyses performed for 37 cases and 125 controls (response 88.5 %). Treatment with DMARD (84 %) was most frequently used, followed by glucocorticoids (66 %) and immunosuppressives (65 %). Twenty percent reported to have ever been taking biologics. Medication use did not differ significantly between cases and controls. Conclusions: Our results did not show an association between medications used and malignancies in JIA patients

Developing an Awareness Campaign to Reduce Second Hand Smoke Among Disadvantaged FamiliesA Participatory M-Health Approach

Children from disadvantaged families are particularly exposed to second hand smoke in their home environment. Using a mixed methods participatory approach, we aimed at identifying suitable media channels and appropriate content for a campaign increasing caregivers' knowledge about the risks of second hand smoke (SHS) exposure for their children and appropriate measures for exposure reduction. In the first phase of the mixed method design, we evaluated knowledge and norms about children's SHS and perceived barriers for avoiding it. To this end, we conducted 26 one-to-one interviews with smoking caregivers of children below the age of six years. Subsequently, we developed and illustrated core messages and identified appropriate communication channels. These were evaluated in focus group discussions by 20 of the 26 interview participants. After a final revision, 121 caregivers evaluated the campaign via an online questionnaire. Online social networks were identified as the most suitable media channel. For these, we developed animated photos with voiceovers addressing the potential consequences of SHS for children. The overall rating of the campaign messages was promising. Participants confirmed that it was important to address the issue in social media. However, sharing the pictures was considered unlikely due to the sensitive topic of the campaign, while the importance of doctors or scientists being recognizable as a source was highlighted. Employing a participatory approach, we developed an m-health campaign, which can now be disseminated in social networks to reach the target population. The effectiveness of the campaign should be evaluated

Education and employment in patients with juvenile idiopathic arthritis - a standardized comparison to the German general population

Background: Although several studies show that JIA-patients have significantly lower employment rates than the general population, the research on educational and occupational attainments in patients with juvenile idiopathic arthritis (JIA) remain conflicting most likely due to small sample sizes. Therefore, aim of this study is to compare the educational achievements and employment status of 3698 JIA-patients with the German general population (GGP). Methods: "SEPIA" was a large cross-sectional study on the current status of a historic cohort of JIA-patients treated in a single center between 1952 and 2010. For the analyses of education and employment a sub-cohort was extracted, including only adult cases with a confirmed diagnosis of JIA (N = 2696). Participants were asked to fill out a standardized written questionnaire on education and employment. Outcome measures (education/unemployment) were directly standardized to the GGP using data obtained from the National Educational Panel Study 2013 (N = 11,728) and the German Unemployment Statistics 2012 of the Federal Statistical Office (N = 42,791,000). Results: After age-and sex-standardization, 3% (95% Confidence Interval 1.9 to 4.1%) more of the JIA-patients (26%) than of the GGP (23%) had only reached primary education. In contrast, parents of JIA-patients had similar levels of education as parents in the GGP. With a standardized difference of 0.2% (95% CI: 0.16 to 0.19%), the unemployment rate in JIA-patients was slightly, but not significantly higher than in the GGP. Stratifying for disease duration and the current treatment status, differences were confirmed for persons diagnosed before 2001, whilst for patients diagnosed after 2000, differences were found only in JIA-patients with ongoing disease. Medium and high educational achievements did not differ statistically significant between JIA patients and the GPP. Conclusion: Educational achievements in German JIA-patients are significantly lower than in the GGP. Furthermore we were able to identify a slightly higher level of unemployment, especially in those with still under treatment and longer disease duration. Better treatment options as well as further development of social support programs might help to overcome this lifelong secondary effect of JIA

Long-Term Health-Related Quality of Life in German Patients with Juvenile Idiopathic Arthritis in Comparison to German General Population

Objective Aims of the study were to investigate health-related quality of life (HRQOL) in adult patients with former diagnosis of Juvenile Idiopathic Arthritis (JIA), to compare their HRQOL with the general population and to identify factors related to a poor outcome. Methods In 2012, a cross-sectional survey was performed by mailing a questionnaire to a large cohort of former and current patients of the German Centre for Rheumatology in Children and Adolescents. Only adult patients (>= 18 years) with a diagnosis compatible with JIA were included (n = 2592;response 66%). The questionnaire included information about HRQOL (EQ5D), disease-related questions and socio-demographics. Prevalence and 95% confidence intervals (CI) of problems with mobility, self-care, usual activities, pain and anxiety/depression were standardized to the German general population. Factors associated with low HRQOL in JIA patients were identified using logistic regression models. Results Sixty-two percent of the study population was female;age range was 18-73 years. In all dimensions, JIA patients reported statistically significantly more problems than the general population with largest differences in the pain dimension (JIA patients 56%;95% CI 55-58%;general population 28%;26-29%) and the anxiety/ depression dimension (28%;27-29% vs. 4%;4-5%). Lower HRQOL in JIA patients was associated with female sex, older age, lower level of education, still being under rheumatic treatment and disability. Conclusions HRQOL in adult JIA patients is considerably lower than in the general population. As this cohort includes historic patients the new therapeutic schemes available today are expected to improve HRQOL in future

Additional file 1: of Association between drug intake and incidence of malignancies in patients with Juvenile Idiopathic Arthritis: a nested case–control study

Intake and duration of rheumatic drugs. (DOCX 28 kb

Health-related quality of life in JIA patients and the general German population.

<p>Age and gender standardized prevalence of problems in the five EQ5D dimensions with 95% confidence intervals.</p