Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis

Funding Information: The author(s) disclosed receipt of the following financial support for the research, authorship and/or publication of this article: the inception cohort project is supported by an unrestricted grant from the Joachim Herz Stiftung, Hamburg, Germany. Publisher Copyright: © The Author(s) 2023.Objectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis. Methods: The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians’ and the patients’ global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models. Results: Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity. Conclusion: Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.Peer reviewe

DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT, ANTIBODY PATTERN AND HAVE SIGNIFICANTLY MORE SEVERE DISEASE IN THE LARGEST JSSC COHORT OF THE WORLD. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Peer reviewe

Cleantech insinöörikoulutuksessa

Cleantech-insinoorit -projekti on Lahden ammattikorkeakoulun tekniikan alan hallinnoima ja Hameen ELY-keskuksen osarahoittama Euroopan Sosiaalirahaston hanke, jonka tavoitteena on parantaa Paijat-Hameen cleantech-osaamista seka insinoorikoulutuksen tyoelamavastaavuutta. Projektin tavoitteena on tuottaa uudenlainen koulutuksen toimintamalli, joka luo aitoja edellytyksia cleantech-innovaatioiden syntymiselle seka uusille tuotteille ja palveluille ymparistoliiketoiminnan alueella. Uuden yhteistyo- ja toimintamallin kehittaminen tekniikan alan koulutukseen perustuu yhteistyohon yrityselaman ja muiden toimijoiden kanssa. Yhteistyota on toteutettu erilaisissa opiskelija- ja T&K-projekteissa, opinnaytetoissa, koulutuksissa, tyopajoissa, seminaareissa, opintomatkoilla ja benchmarkkaamalla erilaisia kaytantoja koti- ja ulkomailla. Uusi yhteistyo- ja toimintamalli kasittaa lisaksi CDIO-/PBL-pedagogiikkaan (CDIO = Conceive – Design – Implement – Operate- / PBL = Problem Based Learning) perustuvan oppimismallin tuomisen tekniikan alan koulutukseen perinteisten insinooritietojen ja -taitojen ohella seka cleantech-osaamisen sisallyttamisen opintosuunnitelmiin. Myos tyoelamalta saadut tiedot tyoelamaosaamisen osaamis- ja muista tarpeista sisallytetaan opintosuunnitelmiin. Lahden ammattikorkeakoulu haluaa osaltaan seka kehittaa insinoorikoulutusta vastaamaan alueen cleantech-yritysten osaamistarpeita etta osallistua alueen yritysten tuotteiden, palvelujen ja prosessien kehittamiseen. Cleantech-insinoorit -projektin tarkoituksena on tunnistaa puhtaan teknologian suunnittelemisessa ja hyodyntamisessa tarvittavia tietoja ja taitoja ja integroida ne insinoorien koulutukseen. Lahden ammattikorkeakoulun tekniikan alalla on viisi koulutusohjelmaa, joista jokaisen cleantech-tarpeita on projektissa tarkasteltu. Lisaksi projektissa selvitettiin olemassa olevan tiedon avulla cleantech-sektorin tarpeita. Cleantech-osaamisen koulutustarvekartoitus tehtiin syksylla 2011, josta tarkempi koulutussuunnitelma cleantech-osaamisen parantamiseksi Paijat-Hameessa valmistui joulukuussa 2011. Kartoituksen ja yritysten haastattelujen pohjalta projektissa toteutettiin cleantech-osaamisen parantamiseksi koulutuksia vuosina 2012 – 2013. Koulutukset pureutuivat ajankohtaisiin teemoihin, kuten vihreaan ICT:hen, energia- ja materiaalitehokkuuteen, kestaviin hankintoihin seka ajankohtaisiin lainsaadannon vaatimuksiin. Tassa artikkelikokoelmassa kerrotaan Lahden ammattikorkeakoulun tekniikan alan koulutusohjelmien toimenpiteista, joilla alueen ja insinoorikoulutuksen cleantech-osaamista ja tyoelamavastaavuutta on parannettu. Raportin suomenkielisessa osassa on tarkasteltu cleantech-liiketoiminnan globaaleja markkinoita ja tulevaisuuden nakymia seka Suomen ja Lahden seudun cleantech-osaamista ja osaamistarpeita. Materiaalitekniikan koulutusohjelman artikkeleissa on kasitelty opintosuunnitelman muutostyota, joka kasittaa myos cleantech-nakokulman ja CDIO- / PBL-pedagogiikan sisallyttamisen opintosuunnitelmaan. Mekatroniikan koulutusohjelman artikkelissa on kasitelty CDIO- / PBL-pedagogiikan lahestymistapaa ja toteutumista kone- ja tuotantotekniikan koulutusohjelmassa. Raportin englanninkielinen osuus kasittelee ymparistoteknologian koulutusohjelman opintosuunnitelman muutosta, joka sisaltaa myos englanninkielisen ylemman ammattikorkeakoulututkinnon. Lisaksi englanninkieliset artikkelit kasittelevat tietotekniikan koulutusohjelman cleantech-nakokulmaa Green ICT -moduuleissa

Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? Clinical characteristics of a juvenile systemic sclerosis cohort

Introduction: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. Methods: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. Clinical characteristics and patient-related outcomes were assessed. Results: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. The mean age at the onset of Raynaud's and first non-Raynaud's symptoms was similar in both groups, approximately 9 and 10.5 years. Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). Discussion: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis

Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 80 Patients at First Assessment

Hamburger Zentrum Kinder & Jugendrheumatol, Hamburg, GermanyGerman Rheumatism Res Ctr, Epidemiol Unit, Berlin, GermanyIstanbul Univ, Cerrahpasa Med Sch, Dept Pediat Rheumatol, Istanbul, TurkeyUniv Childrens Hosp, Riga, LatviaUniv Fed Sao Paulo, Pediat Rheumatol Unit, Sao Paulo, BrazilSci Ctr Childrens Hlth, Rheumatol, Moscow, RussiaHosp Pediat Prof Dr Juan P Garrahan, Serv Immunol & Rheumatol, Buenos Aires, DF, ArgentinaUniv Firenze, Pediat, Florence, ItalyState Pediat Med Univ, Hosp Pediat, St Petersburg, RussiaHosp Special Surg, Div Ped Rheum PTD, 535 E 70th St, New York, NY 10021 USAUniv Hosp Ramon y Cajal, Dept Rheumatol, Madrid, SpainUniv Ghent, Ghent Univ Hosp, Dept Rheumatol, Ghent, BelgiumUniv Fed Rio de Janeiro, Rio De Janeiro, BrazilUniv Childrens Hosp, Ljubljana, SloveniaHosp Garcia de Orta, Dept Rheumatol, Rheumatol, Almada, PortugalGen Univ Hosp Prague, Pediat Rheumatol Unit, Dept Pediat & Adolescent Med, Prague, Czech RepublicHosp Ninos Dr Orlando Alasia, Santa Fe, ArgentinaGreat Ormond St Hosp Sick Children, Paediat Rheumatol Dept, London, EnglandSchneider Childrens Med Ctr Israel, Pediat Rheumatol Unit, Petah Tiqwa, IsraelCharite Univ Med Berlin, Berlin, GermanyHosp St Joan de Deu, Unitat Reumatol Pediat, Barcelona, SpainRigshosp, Copenhagen, DenmarkUniv Pittsburgh, Med Ctr, Pediat Rheumatol, Pittsburgh, PA USAMeir Med Ctr, Kefar Sava, IsraelHamburg Ctr Pediat & Adolescent Rheumatol, Hamburg, GermanyPediatric Rheumatology Unit, Universidade Federal de São Paulo (UNIFESP), São Paulo, BrazilWeb of Scienc