The national CAPS/TRAPS/MKD/HIDS expert working group was established, defined its aims and conducted a comprehensive literature review synthesising the recent (2013 to 2018) published evidence including all available recommendations for diagnosis and management. CAS  The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. This has facilitated impactful treat-to-target strategies resulting in better outcomes [55]. Das Zentrum für pädiatrische Rheumatologie ist Teil des Tübinger pädiatrischen Inflammationszentrums. HNO-Heilkunde. Most respondents reported a negative influence of the COVID-19 pandemic on their current life situation. More recently, 10-year safety data of IL-1 inhibition has become available for CAPS [47]. Overall, continuity of care was associated with higher patient satisfaction in various categories (Supplement 2). 1 72076 Tübingen 07071 29-83781 Kliniken Kinderklinik Kinderheilkunde I Zentrum für pädiatrische Rheumatologie Zentrum für pädiatrische Rheumatologie Das Zentrum für pädiatrische Rheumatologie ist Teil des Tübinger pädiatrischen Inflammationszentrums. Kim S, Kahn P, Robinson AB, Lang B, Shulman A, Oberle EJ, et al. 1 this recommendation was noticeably modified in comparison with the SHARE recommendations.2 regular checks should also be carried out with low or absent disease activity (see treat-to-target). Ann Rheum Dis. Sie können bei uns auch einen Termin zur Videosprechstunde vereinbaren, damit Sie, um Ansteckung zu vermeiden, nicht in die Praxis kommen müssen. After excluding all participants in remission, the drop-out rate was 4.7%. Ärztlicher Leiter Dr. Sebastian Saur Koordinator 07071 29-84095 07071 29-2763 sebastian.saur@med.uni-tuebingen Measures of HRQoL and disease damage should be integrated into care [28, 29]. 1 Garmisch-Partenkirchen, Oktober 2006 Gastschriftleiter › Author Affiliations. 2017;8:1410. 2010;28(5):790–7. Finde jetzt die besten Unternehmen mit echten Bewertungen und hilfreichen Tipps der golocal Community. Eur J Pediatr. Respondents indicated a high patient satisfaction, although it decreased after transfer to adult care. Entry criteria for distinct levels of disease activity, risk factors for adverse outcomes and sensible timelines for evaluating outcomes have to be defined. 2018;378(20):1908–19. Apart from patients in remission, 4.7% discontinued treatment in adult medicine due to referral problems. Overall, this study found high follow-up rates in the cohort within the TTP, while 21.2% of patients reported not seeing an adult rheumatologist regularly. 3b). The reported average duration of physical activity per week was 3.4 hours (SD 2.9) and ranged from zero to 14 hours per week. Google Scholar. Higher scores on the EQ VAS and lower EQ-5D-5L index scores indicate better health-related quality of life, lower PGA scores indicate less disease activity. In parallel, the progress of the PRO-KIND working group was presented and discussed at the annual GKJR Scientific Meetings 2016–2018. Important guiding principles were established; optimal treatment selection and monitoring concepts were defined. High PGA scores at the time of referral and low physical activity correlated with lower long-term HR-QoL. Pediatr Rheumatol Online J. A molecular genetic confirmation of a rare AID is desirable, yet not always possible. Heijstek MW, Ott de Bruin LM, Borrow R, van der Klis F, Kone-Paut I, Fasth A, et al. 2015;11(10):1083–92. Additional suggestions were made for particularly severe disease. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. AIDAI: AutoInflammatory Diseases Activity Index [26]; ADDI: Autoinflammatory Disease Damage Index for assessment of the extent of organ damage [29]; PGA and PPGA: Physician and patient global assessment using a visual analogue scale. 2011;70(10):1704–12. In three cases, a stable remission occurred, leaving them with the diagnosis of “condition after” their initial pediatric rheumatic diagnosis. Transfer in this respect is the final step of the transition process. In addition typical clinical symptoms for CAPS include urticaria-like rash, cold/stress triggered episodes, sensorineural hearing loss, chronic aseptic meningitis and skeletal abnormalities such as epiphyseal overgrowth and frontal bossing [11]. Of the 65 participants who initially received continued care, 12.3% (8/65) reported that they were seeing their rheumatologist less than once a year, 15.4% (10/65) reported a yearly, 36.9% (24/65) a six-monthly frequency of visits, while 33.8% (23/65) reported four or more visits per year. The dataset generated and analyzed during this study is not publicly available but is available from the corresponding author on reasonable request after obtaining ethics committee approval. Short form to Abbreviate Biologika In Der Kinderrheumatologie. In every instance, lower satisfaction was associated with lower scores in the EQ-5D-index and the EQ VAS (Table 3a). c Each column represents 100% of participants either overall or in the respective age group. Furthermore, the TTP tries to individualise the timing of transfer in close cooperation with patients, parents, the medical team, and psycho-social workers according to patients’ transfer readiness and disease activity. The impact of the COVID-19 pandemic on trajectories of well-being of middle-aged and older adults: a multidimensional and multidirectional perspective. Pediatric Rheumatol. 2011;70(12):2155–8. Treatment discontinuation rates were low and mostly due to remission. 2014;73(12):2168–73. 2012;64(7):2375–86. The health-related quality of life (HR-QoL) was assessed by the EQ-5D-5L, a validated instrument measuring the HR-QoL in five dimensions on a five-level Likert scale with equal spacing: mobility, self-care, daily activities, pain/discomfort, and anxiety/depression (1: none, 2: slight, 3: moderate, 4: severe, 5: extreme problems/unable to accomplish) [14]. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work. Sandra Hansmann. 2018;16(1):7. Schönklinikum Eilbek Dehnhaide 120, Haus 8 22081 Hamburg, Germany. S, strength of recommendation: A, consistent level 1 studies; B, consistent level 2 or 3 studies or extrapolations from level 1 studies; C, level 4 studies or extrapolations from level 2 or 3 studies; D, level 5 evidence or troublingly inconsistent or inconclusive studies of any level. This study was carried out in compliance with the Declaration of Helsinki and approved by the ethics committee of the Faculty of Medicine, University of Tuebingen (411/2020B0). We provide excellent research and teaching aimed at finding solutions to future challenges in a globalized society. Kuemmerle-Deschner JB, Koitschev A, Tyrrell PN, Plontke SK, Deschner N, Hansmann S, et al. 2017;76(6):942–7. 2020;79(7):649–59. Levy R, Gerard L, Kuemmerle-Deschner J, Lachmann HJ, Kone-Paut I, Cantarini L, et al. Google Scholar. In accordance with Stringer et al., patient satisfaction scores > 7 were defined as “high”, ≤7 and ≥ 5 as “moderate” and <  5 as “low satisfaction” [18]. Werdegang. Statements were integrated into diagnosis- and disease activity specific treat-to-target CTPs for CAPS, TRAPS and MKD/HIDS. Fachärztin für Kinder- und Jugendmedizin Universitätsklinikum Tübingen. autoinflammation@med.uni-tuebingen.de, Das Zentrum deckt ein breites Spektrum unterschiedlicher, z. T, äußerst seltener Erkrankungen ab, Um eine optimale Patientenversorgung zu gewährleisten, arbeiten wir auch mit anderen Zentren zusammen und tauschen uns auf unterschiedlichen Ebenen intensiv aus, Zertifizierung von überregionalen EMAH-Zentren & EMAH-Schwerpunkten, Qualitätssicherung für die stationäre Versorgung von Kindern und Jugendlichen, Deutsches Herzkompetenz Zentrum am Universitätsklinikum Tübingen, Kooperation für Transparenz und Qualität im Gesundheitswesen, Die Altersvorsorge für den Öffentlichen Dienst, © 2023 Universitätsklinikum und Medizinische Fakultät Tübingen. a We ranked basic therapy as level 1, biologicals and JAK-inhibitors as level 2, and steroids as level 3. Real-life effectiveness of canakinumab in cryopyrin-associated periodic syndrome. S.H. From these scores, a personal health state as well as an index-value can be derived. 2021;18(22):12196. This study contributes to further optimize transition and promote long-term health of pediatric rheumatology patients, measuring individual, social and health care outcomes. Other team members such as physiotherapists and teachers are also involved. Legend: Listed are the significant differences in patient satisfaction between participants that did experience some form of discontinuation of care, including change in diagnosis, medical therapy or discontinuation in general. 3a). Article  71.8% of participants (61/85) reported no hospitalisations after leaving the TTP. 2015;13(1):43. In addition, 27 participants (27/65; 41.5%) reported that their prescribed medication was changed during adult care. Piram M, Kone-Paut I, Lachmann HJ, Frenkel J, Ozen S, Kuemmerle-Deschner J, et al. Ann Rheum Dis. Email: sprechstunde@kinderrheumatologie.de . 2013;65(3):824–31. Rare autoinflammatory diseases (AIDs) including Cryopyrin-Associated Periodic Syndrome (CAPS), Tumor Necrosis Receptor-Associated Periodic Syndrome (TRAPS) and Mevalonate Kinase Deficiency Syndrome (MKD)/ Hyper-IgD Syndrome (HIDS) are genetically defined and characterized by recurrent fever episodes and inflammatory organ manifestations. Kullenberg T, Lofqvist M, Leinonen M, Goldbach-Mansky R, Olivecrona H. Long-term safety profile of anakinra in patients with severe cryopyrin-associated periodic syndromes. This resulted in 38 drafted statements, of which two were removed due to the lack of consensus. 2019;78:1025–32. PubMed Google Scholar. Furthermore, the importance of physical activity for the long-term HR-QoL of rheumatologic patients seems to be supported in this cohort. 2016;68(11):2795–805. Privacy Continuity of care. Only two participants reported problems with referral that led to the discontinuation of rheumatological care (Fig. 2014;73(12):2160–7. Ausführliche Informationen zum Aufenthalt in unserer Klinik finden Sie auf der Seite Klinikaufenthalt. Kostjukovits S, Kalliokoski L, Antila K, Korppi M. Treatment of hyperimmunoglobulinemia D syndrome with biologics in children: review of the literature and Finnish experience. Overall, HR-QoL was high (79.8 on the EQ VAS), yet lower than in the average population. The PRO-KIND CAPS/TRAPS/MKD/HIDS CTP approach follows the model set by Swart [55] and Consolaro [56] for JIA. Address of Kinderarztpraxis Ingelheim am Rhein - Kinderrheumatologie - Neonatologie is Marktpl. Patients who did not consent to participation or did not respond were excluded. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Sustained remission of symptoms and improved health-related quality of life in patients with cryopyrin-associated periodic syndrome treated with canakinumab: results of a double-blind placebo-controlled randomized withdrawal study. Article  Ann Rheum Dis. Ihre Haus- & Fachärzte von der Geburt bis zum vollendeten 18. For one aspect, it can be assumed that patients who had a better experience in the TTP might have been more likely to respond to the invitation to participate in this study than those who have no good memories of their time in the TTP. 2009;48(8):987–91. [10] have described a decline in overall patient satisfaction after the transfer to adult care, which was observed to a lesser extent in this cohort. Russo RA, Melo-Gomes S, Lachmann HJ, Wynne K, Rajput K, Eleftheriou D, et al. a HR-QoL compared to the average German population. PRO-KIND advances the standardization of diagnosis and management of childhood rheumatic diseases. Most importantly, high-level evidence from a randomized controlled trial for the effectiveness of IL-1 inhibition for TRAPS and MKD/HIDS was integrated [27]. Validation of the auto-inflammatory diseases activity index (AIDAI) for hereditary recurrent fever syndromes. They are divided into participants who reported weekly physical activity of 2.5 hours or more (lower dark bar) and those who reported less weekly physical activity (upper light bar). b Participation in organized club. 2009;47(2):255–61. The speaker (JKD) and the coordinator (EL) were selected. Jaeger VK, Hoffman HM, van der Poll T, Tilson H, Seibert J, Speziale A, et al. Article  Methods for Analysing and reporting EQ-5D data. Bis heute 11 Jahre und 8 Monate, seit Okt. Your privacy choices/Manage cookies we use in the preference centre. The PRO-KIND CAPS/TRAPS/ MKD/HIDS expert working group adjusted these in iterative reviews. König H-H, Bernert S, Angermeyer MC, Matschinger H, Martinez M, Vilagut G, et al. Article  2019. p. 1 online resource (x, 820 pages). * The PRO-KIND statements were adapted from the SHARE recommendations for the management of autoinflammatory diseases [19]. Safety of vaccinations in patients with cryopyrin-associated periodic syndromes: a prospective registry based study. 2017;76(11):1788–96. Kinderrheumatologie. Consolaro A, Ruperto N, Bazso A, Pistorio A, Magni-Manzoni S, Filocamo G, et al. Correspondence to Ann Rheum Dis. The European network for care of children with paediatric rheumatic diseases: care across borders. Copyright © 2019 Kinderarztpraxis Neustadt - Kinderarzt in Luzern. 2014;41(2):345. Allgemeine & Innere Medizin | The best possible preparation of patients is required to ensure a successful transfer and improved long-term quality of life. https://doi.org/10.1186/s12969-020-0409-3, DOI: https://doi.org/10.1186/s12969-020-0409-3. Rheumatology (Oxford). 2011;11(2):112–22. International and interdisciplinary identification of health care transition outcomes. 2019;71(6):846–63. The PRO-KIND working group for the rare hereditary autoinflammatory syndromes CAPS, TRAPS and MKD/HIDS was established in February 2015. The majority of the study cohort reported no difficulties in each category of the EQ-5D-5L while three out of four participants indicated no more than slight problems in any category. Google Scholar. Legend: a Descriptive results on continuity of care. Lane T, Loeffler JM, Rowczenio DM, Gilbertson JA, Bybee A, Russell TL, et al. Many programs analyze satisfaction with the transition process as the main goal [8,9,10,11], but the focus of such programs is on long-term health and care improvement. Publication of this manuscript was supported by the Open Access Publishing Fund of the University of Tuebingen. J.B.K.-D. performed clinical studies with and received educational, research and centre grants and honoraria from Novartis, AbbVie and SOBI. Demographic characteristics of the study cohort included age, gender, marital status, living situation and levels of education and employment. http://www.cebm.net/oxford-centre-evidence-based-medicine-levels-evidence-march-2009/: Center for evidence-based medicine; March 2009 [01.10.2019]. Therefore, all treatment decisions follow the treat-to-target concept and enable precision health care for children. 1) The PRO-KIND CAPS/TRAPS/MKD/HIDS expert working group consisted of only 12 members, which limited the scale and scope of contributing experiences. 2022;23(7):3577–604. These provide a path to a rapid evaluation, effective control of disease activity and tailored adjustment of therapies. These charts show the number of patients with a discontinuation of care after initial transfer to adult rheumatology, reasons for discontinuation of care, changes in diagnosis or medication after transfer and the current frequency of visits to adult rheumatology. Neben der Allgemeinen Kinderheilkunde werden hier vor allem Kinder und Jugendliche mit Krebserkrankungen behandelt. 1 vote What is the abbreviation for Biologika In Der Kinderrheumatologie? 2016;11(4):e0153267. Of the 65 participants who reported continuation of care after the transfer, eight stated that care had been disrupted afterwards. The definition of therapeutic targets, the continuous integration of disease activity measurements and the clinical evaluation at defined time points are the basis for the German PRO-KIND CTPs. There are several limitations to the study. Reasons for hospitalisations relevant to this study were repeated inflammatory episodes, joint (knee and shoulder) and ophthalmologic complications. [35], who also reported a significantly lower transfer rate for patients in remission. The Oxford Centre for Evidence-based Medicine levels of evidence and grades of recommendation were utilized to support the evidence for each statement [24]. Autoinflammation reference center Tuebingen, Arthritis and Rheumatology Documentation and Information System; axaris - software & systeme GmbH, Studie zur Gesundheit Erwachsener in Deutschland, European Alliance of Associations for Rheumatology, Center for Interdisciplinary Clinical Immunology, Rheumatology and Autoinflammatory Diseases. In contrast, there is a very mild AID phenotype with limited disease activity: some CAPS patients experience significantly fewer symptoms during warm seasons, TRAPS patients may have very infrequent episodes of clinically active disease [60]. The criteria for evaluating long-term outcomes in transition are complex constructs, and often validated measurement tools in the patients’ language are lacking. Methods: In May 2020 all GKJR members were invited to take part in an online survey. The phenotype of TNF receptor-associated autoinflammatory syndrome (TRAPS) at presentation: a series of 158 cases from the Eurofever/EUROTRAPS international registry. These had synthesized the published evidence until June 2013. Disease subtype, risk factors at onset and disease activity are the foundation for the traditional stratified ACR juvenile idiopathic arthritis (JIA) treatment recommendations [49, 50]. The research was conducted ethically in accordance with the World Medical Association Declaration of Helsinki. Stuttgart Zentrum für Rheumatologie am Klinikum Stuttgart (ZEPRAS) Olgahospital, Pädiatrie 2 Kriegsbergstraße 62 70174 Stuttgart Dr. med. At diagnosis, the patient’s individual disease activity in addition to the overall AID diagnosis should guide initial management decisions. Rheumatol Int. Muehlan H, Markwart H, Menrath I, Ernst G, Thyen U, Schmidt S. A short form of the child/youth health care questionnaire on satisfaction, utilization, and needs in children and adolescents with a chronic condition (CHC-SUN-SF/YHC-SUN-SF). J Adolesc Health. The longer version of this instrument unfortunately did not seem fitting for this study since it surveyed topics that do not apply to the process and cohort of the TTP, e.g., working environment. McDonagh JE, Southwood TR, Shaw KL, BSPAR. The relationship between physical activity levels and pain in children with juvenile idiopathic arthritis. Development and preliminary validation of the ‘mind the gap’ scale to assess satisfaction with transitional health care among adolescents with juvenile idiopathic arthritis. PubMed Google Scholar. Correspondence to Bitte melden Sie sich über Ihr Handy in unserer zertifizierten PraxisApp des Berufsverbandes der Kinder-und Jugendärzte an (Link zur Praxis App siehe oben rechts) und betreten dann dort zum vereinbarten Termin das virtuelle Wartezimmer. Dipl. The effectiveness of treat-to-target studies with individual dose adjustments bares the promise of excellent control of disease activity [17]. a Frequency of physical activity. Arthritis Rheum. Patients usually start in the TTP at age thirteen, depending on their individual maturity and disease activity. Google Scholar. Research Topic Children's Rheumatism. Evidence-based PRO-KIND diagnostic algorithm for CAPS/TRAPS/MKD/HIDS. Google Scholar. Concerning their experience in adult care, 86.1% (56/65) declared that they had enough time to ask all their individual questions and 84.6% (55/65) stated that they received sufficient and intelligible answers. Hinze CH, Oommen PT, Dressler F, Urban A, Weller-Heinemann F, Speth F, et al. Evidence levels were adapted from the Oxford Centre for Evidence-based Medicine levels of evidence and grades of recommendation [24]: 1A, Systematic reviews of randomized controlled trials; 1B, individual randomised controlled trial; 2A, systematic review of cohort studies; 2B, individual cohort study; 3B, individual case-control study, non-consecutive cohort study; 4, case series; 5, expert opinions. Minden K. Adult outcomes of patients with juvenile idiopathic arthritis. Über die Praxis App erhalten Sie auch  Informationen zur Praxis und die Information über anstehende Vorsorgeuntersuchungen und Impfungen. Pediatric Rheumatology Aufgrund der langjährigen Ausbildung zum Kinderrheumatologen (Universitätsklinik Tübingen, Deutschland) und der Sprechstundenerfahrung in einem grossen Referenzzentrum für autoinflammatorische Erkrankungen, stehen wir Ihnen in diesem Spezialgebiet für Diagnostik, Beratung, Zweitmeinungen und Therapie zur Verfügung. 410 Hoppe-Seyler-Str. Rheumatology. Efficacy of etanercept in the tumor necrosis factor receptor-associated periodic syndrome: a prospective, open-label, dose-escalation study. 2013;15(1):R33. Orphanet J Rare Dis. 2003;48(3):767–75. Toward a treat-to-target approach in the management of juvenile idiopathic arthritis. Entwicklungsneurologie (2016) Hashkes PJ, Laxer RM, Simon A, Ohio Library and Information Network. Blank N, Schönland SO. Boeker, L.S., Kuemmerle-Deschner, J.B., Saur, S.J. Sustained response and prevention of damage progression in patients with neonatal-onset multisystem inflammatory disease treated with anakinra: a cohort study to determine three- and five-year outcomes. Toplak N, Frenkel J, Ozen S, Lachmann HJ, Woo P, Kone-Paut I, et al. Arthritis Rheum. Long-term outcome of a successful cord blood stem cell transplant in mevalonate kinase deficiency. Kinderarztpraxis Ingelheim am Rhein - Kinderrheumatologie - Neonatologie can be contacted at 4961328998444. In addition, the risk of organ damage may differ dramatically between AID patients as demonstrated for the risk of hearing loss in CAPS [40]. Ann Rheum Dis. 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