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Sökning: onr:"swepub:oai:DiVA.org:uu-496022" > Differences Sustain...

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort

Foeldvari, Ivan (författare)
Schon Klin Hamburg Eilbek, Hamburg Ctr Pediat & Adolescent Rheumatol, Hamburg, Germany.
Klotsche, Jens (författare)
German Rheumatism Res Ctr, Berlin, Germany.
Kasapcopur, Ozgur (författare)
Istanbul Univ Cerrahpasa, Cerrahpasa Med Sch, Istanbul, Turkey.
visa fler...
Adrovic, Amra (författare)
Istanbul Univ Cerrahpasa, Cerrahpasa Med Sch, Istanbul, Turkey.
Terreri, Maria Teresa (författare)
Univ Fed Sao Paulo, Sao Paulo, Brazil.
Sakamoto, Ana Paula (författare)
Univ Fed Sao Paulo, Sao Paulo, Brazil.
Stanevicha, Valda (författare)
Riga Stradins Univ, Univ Children Hosp, Riga, Latvia.
Sztajnbok, Flavio (författare)
Univ Estado, Rio De Janeiro, Brazil.
Anton, Jordi (författare)
Univ Barcelona, Esplugues, Hosp St Joan de Deu, Barcelona, Spain.
Feldman, Brian (författare)
Hosp Sick Children, SickKids, Toronto, ON, Canada.
Alexeeva, Ekaterina (författare)
Sechenov First Moscow State Med Univ, Minist Hlth Russian Federat, Natl Med Res Ctr Childrens Hlth, Moscow, Russia.
Katsicas, Maria (författare)
Hosp Pediat JP Garrahan, Buenos Aires, DF, Argentina.
Smith, Vanessa (författare)
Univ Ghent, Ghent, Belgium.;Ghent Univ Hosp, Ghent, Belgium.
Avcin, Tadej (författare)
Univ Med Ctr Ljubljana, Univ Childrens Hosp, Ljubljana, Slovenia.
Marrani, Edoardo (författare)
Meyer Childrens Hosp, Florence, Italy.
Kostik, Mikhail (författare)
St Petersburg State Pediat Med Univ, St Petersburg, Russia.
Lehman, Thomas (författare)
Hosp Special Surg, 535 E 70th St, New York, NY 10021 USA.
Sifuentes-Giraldo, Walter-Alberto (författare)
Univ Hosp Ramon y Cajal, Madrid, Spain.
Vasquez-Canizares, Natalia (författare)
Childrens Hosp Montefiore, Bronx, NY USA.
Appenzeller, Simone (författare)
Univ Estadual Campinas, Sch Med Sci, Sao Paulo, Brazil.
Janarthanan, Mahesh (författare)
Sri Ramachandra Univ, Chennai, Tamil Nadu, India.
Moll, Monika (författare)
Univ Tubingen, Tubingen, Germany.
Nemcova, Dana (författare)
Charles Univ Prague, Prague, Czech Republic.
Patwardhan, Anjali (författare)
Univ Missouri, Columbia, MO 65211 USA.
Santos, Maria Jose (författare)
Hosp Garcia de Orta, Almada, Portugal.
Sawhney, Sujata (författare)
Sir Ganga Ram Hosp, Delhi, India.
Schonenberg-Meinema, Dieneke (författare)
Univ Amsterdam, Amsterdam Univ Med Ctr, Emma Childrens Hosp, Amsterdam, Netherlands.
Battagliotti, Cristina (författare)
Hosp Ninos Dr Orlando Alassia, Santa Fe, Argentina.
Berntson, Lillemor, 1957- (författare)
Uppsala universitet,Pediatrisk inflammations- och metabolismforskning samt barnhälsa
Bica, Blanca (författare)
Hosp Univ Clementino Fraga Filho, Rio De Janeiro, Brazil.;Univ Fed Rio de Janeiro, Rio De Janeiro, Brazil.
Brunner, Jurgen (författare)
Med Univ Innsbruck, Innsbruck, Austria.
Costa-Reis, Patricia (författare)
Univ Lisbon, Hosp Santa Maria, Lisbon, Portugal.
Eleftheriou, Despina (författare)
Great Ormond St Hosp Children NHS Fdn Trust, London, England.
Harel, Liora (författare)
Tel Aviv Univ, Fac Med, Schneider Childrens Med Ctr Israel Sackler, Petah Tiqwa, Israel.
Horneff, Gerd (författare)
Asklepios Kln St Augustin, St Augustin, Germany.
Kaiser, Daniela (författare)
Kinderspital, Luzerner Kantonsspital, Luzern, Switzerland.
Kallinich, Tilmann (författare)
Charite, Berlin, Germany.;German Rheumatism Res Ctr Berlin, Berlin, Germany.
Lazarevic, Dragana (författare)
Univ Nis, Clin Ctr Nis, Nish, Serbia.
Minden, Kirsten (författare)
Charite, Berlin, Germany.;German Rheumatism Res Ctr Berlin, Berlin, Germany.
Nielsen, Susan (författare)
Rigshosp, Copenhagen, Denmark.
Nuruzzaman, Farzana (författare)
Stony Brook Childrens Hosp, Stony Brook, NY USA.
Uziel, Yosef (författare)
Tel Aviv Univ, Meir Med Ctr, Tel Aviv, Israel.
Helmus, Nicola (författare)
Schon Klin Hamburg Eilbek, Hamburg Ctr Pediat & Adolescent Rheumatol, Hamburg, Germany.
Torok, Kathryn S. (författare)
Univ Pittsburgh, Childrens Hosp Pittsburgh, Pittsburgh, PA 15224 USA.
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Schon Klin Hamburg Eilbek, Hamburg Ctr Pediat & Adolescent Rheumatol, Hamburg, Germany German Rheumatism Res Ctr, Berlin, Germany. (creator_code:org_t)
2022-06-08
2022
Engelska.
Ingår i: Arthritis care & research. - : John Wiley & Sons. - 2151-464X .- 2151-4658. ; 74:10, s. 1575-1584
  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
Stäng  
  • Objective To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. Methods A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. Results At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. Conclusion Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)

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