Update on juvenile systemic sclerosis

Current Rheumatology Reports
Ivan Foeldvari

Abstract

Juvenile systemic sclerosis is an orphan disease of the macro- and microvasculature with an unknown etiology. Although pediatric and adult classification systems have been developed, neither has been validated yet. Recent publications have shown a shift in the subset pattern in juvenile-onset adult patients, with a better outcome in these patients as compared with young adult patients. As a first step in assessing activity, damage, and severity, a pediatric severity score--a modification of the adult Medsger scale--was proposed but has not been validated. An activity score also has been developed but has not yet been published. Currently, treatment approaches are based only on adult data, and problems exist in extrapolating these data to a pediatric population.

References

Dec 4, 2004·Current Opinion in Rheumatology·M Bashar Kahaleh
Nov 30, 2006·Arthritis and Rheumatism·Giorgia MartiniUNKNOWN Juvenile Scleroderma Working Group of the Pediatric Rheumatology European Society
Mar 17, 2007·Seminars in Arthritis and Rheumatism·Marie HudsonMurray Baron
Sep 26, 2007·Annals of the Rheumatic Diseases·D KhannaUNKNOWN Scleroderma Clinical Trials Consortium co-authors
Jan 17, 2009·Annals of the Rheumatic Diseases·O Kowal-BieleckaUNKNOWN EUSTAR Co-Authors
Mar 2, 2010·Arthritis Care & Research·Ariane L HerrickEileen M Baildam
Sep 17, 2010·The Journal of Rheumatology·Ivan FoeldvariChristopher P Denton
Jun 22, 2011·Rheumatology International·Hadi PoormoghimNeda Toofaninjed
Apr 3, 2012·Seminars in Arthritis and Rheumatism·Kyle M WalkerUNKNOWN Canadian Scleroderma Research Group (CSRG)
Aug 14, 2012·Arthritis and Rheumatism·Francesco La TorreFrancesco Zulian
Sep 27, 2012·Rheumatology International·Afsha A Topal, Rachita S Dhurat
Dec 21, 2012·Annals of the Rheumatic Diseases·Muriel ElhaiUNKNOWN EUSTAR (EULAR Scleroderma Trials and Research group)
May 15, 2013·Arthritis and Rheumatism·Marco Matucci-CerinicFredrick M Wigley
Sep 3, 2013·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Andreas ReiffRobertson Parkman
Oct 31, 2013·Expert Review of Clinical Immunology·Flavia V Castelino, John Varga
Nov 5, 2013·Rheumatology·Susanna CappelliMarco Matucci-Cerinic
Dec 3, 2013·European Respiratory Review : an Official Journal of the European Respiratory Society·Johannes P SchwaigerJ Gerry Coghlan
Dec 18, 2013·Annals of the Rheumatic Diseases·Francesca IngegnoliLuigi Meroni Pier
Dec 20, 2013·The New England Journal of Medicine·Lenny van BonTimothy R D J Radstake
Feb 28, 2014·Arthritis & Rheumatology·Doron RimarZahava Vadasz
Jul 25, 2014·JAMA : the Journal of the American Medical Association·Jacob M van LaarUNKNOWN EBMT/EULAR Scleroderma Study Group

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Citations

Apr 3, 2018·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Andy C Hsi, Ilana S Rosman
Oct 28, 2020·Rheumatology International·Amra AdrovicOzgur Kasapcopur
Jul 27, 2021·Orphanet Journal of Rare Diseases·Eric HachullaUNKNOWN Collaborators

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