Is the Presentation and Severity Different of the Juvenile Diffuse and Limited Subtype Systemic Sclerosis? Results of Juvenile Scleroderma Inception Cohort

Ivan Foeldvari¹, Jens Klotsche ², Ozgur Kasapcopur ³, Amra Adrovic ⁴, Kathryn Torok ⁵, Valda Stanevicha ⁶, Maria Teresa Terreri ⁷, Flavio Sztajnbok ⁸, Ekaterina Alexeeva ⁹, Jordi Antón ¹⁰, Brian Feldman ¹¹, Maria Katsicas ¹², Vanessa Smith ¹³, Tadej Avcin ¹⁴, Rolando Cimaz ¹⁵, Mikhail Kostik ¹⁶, Thomas Lehman ¹⁷, Walter Sifuentes-Giraldo ¹⁸, Natalia Vasquez-Canizares ¹⁹, Simone Appenzeller ²⁰, Mahesh Janarthanan ²¹, Monika Moll ²², Dana Nemcova ²³, Maria José Santos ²⁴, Dieneke Schonenberg ²⁵, Christina Battagliotti ²⁶, Lillemor Berntson ²⁷, Blanca Bica ²⁸, Juergen Brunner ²⁹, Patricia Costa Reis ³⁰, Despina Eleftheriou ³¹, Liora Harel ³², Gerd Horneff ³³, Tilmann Kallinich ³⁴, Dragana Lazarevic ³⁵, Kirsten Minden ³⁶, Susan Nielsen ³⁷, Farzana Nuruzzaman ³⁸, Anjali Patwardhan ³⁹, Yosef Uziel ⁴⁰, Daniela Kaiser ⁴¹ and Nicola Helmus ¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, ⁴Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁵UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁶Riga Stradins University, Riga, Latvia, ⁷Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹National Medical Research Center of Children`s Health, Moscow, Russia, ¹⁰Hospital Sant Joan de Déu, Barcelona, Spain, ¹¹University of Toronto & The Hospital for Sick Children, Toronto, ON, Canada, ¹²Hospital de Pediatria, Buenos Aires, Argentina, ¹³Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, ¹⁴Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁵University Hospital Meyer, Florence, Italy, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁷Hospital for Special Surgery, New York, ¹⁸Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁹Children’s Hospital at Montefiore, Bronx NY, ²⁰UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, ²¹Sri Ramachandra University, Chennai, India, ²²University of Tuebingen, Tuebingen, Germany, ²³General University Hospital, Prague, Czech Republic, ²⁴Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ²⁵Amsterdam UMC, Amsterdam, Netherlands, ²⁶Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁷Uppsala University Hospital, Uppsala, Sweden, ²⁸Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁹Tirol Kliniken, Innsbruck, Austria, ³⁰Hospital de Santa Maria, Lisbon, Portugal, ³¹Great Ormond Street Hospital, London, United Kingdom, ³²Tel Aviv University, Tel Aviv, Israel, ³³Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³⁴Charite, Berlin, Germany, ³⁵KC Niš, Niš, Serbia, ³⁶German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, ³⁷Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, ³⁸Stony Brook Children's Hospital, Stony Brook, NY, ³⁹University of Missouri, Columbia, ⁴⁰Meir Medical Center, Kfar Saba, Israel, ⁴¹Luzerner Kantonsspital | Kinderspital, Luzern, Switzerland

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Juvenile sclerosis, systemic sclerosis and outcome measures

Session Information

Date: Sunday, November 10, 2019

Title: Pediatric Rheumatology – ePoster I: Basic Science, Biomarkers, & Sclerodermic Fever

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with an estimated prevalence of 1 in 1 000 000 children. In the adult systemic scleroderma population there are large differences regarding organ pattern and severity between diffuse and limited subtypes.

Methods: We reviewed all patients of juvenile scleroderma inception cohort (jSScC) at the time of inclusion till 15^thMay 2019. The jSScC is a cohort, where patients, who fulfill the adult 2013 classification criteria , are age under 18 at the time of inclusion and developed the first non-Raynaud before the age of 16 years are included.

Results: 131 patients were included, 72.5% with diffuse subtype. 75% females in the diffuse (djSSc) and 71% in the limited subtype(ljSSc). 86% of patients were Caucasian. Mean age of onset of Raynauds was 9.7 years in the djSSc and 10.7 years in the ljSSc (p=0.8). Mean age of onset of the non-Raynauds was 9.9 years in the djSSc and 11.2 years in the ljSSc (p=0.7). Mean disease duration at time of inclusion was 3.4 years in the djSSc and 2.4 years in the ljSSc There was no significant difference in the ANA, anti-Scl-70 and anticentromere positivity. The mean modified skin score was significantly higher in the djSSc (17.3 compared 7.1, (p=0.3)). They were significantly more teleangiectasia in the djSSc group (39% compared to 19% (p=0.003)). Cardiac involvement was significantly higher in the ljSSc group (19% compared to 3% (p=0.005) There was no significant difference in the proportion of ILD, pulmonary hypertension, gastrointestinal involvement and renal involvement. No renal hypertension was observed. There was significantly more muscle weakness observed in the ljSSc group (38% compared to 17% (p=0.029)). There was no significant difference regarding number of joints with contractions. Physician rated disease activity (40 compared to 29, on 100 mm VAS scale (p=0.013)) and disease damage (37 compared 18, on a 100 mm VAS scale (p< 0.001)) was significantly higher in the djSSc. This significant difference was not found in rating of patients of disease damage and activity. Conclusion: ljSSc and djSSc seems to be more similar than in adult patient with these subtypes, although physician rating of disease activity and damage found the djSSc more severe.

Supported by the “Joachim Herz Stiftung”

Disclosure: I. Foeldvari, Beyer, 5, BMS, 5, Glaxo, 5, Inventa, 5, Novartis, 5; J. Klotsche, None; O. Kasapcopur, AbbVie, 2; A. Adrovic, None; K. Torok, None; V. Stanevicha, None; M. Terreri, None; F. Sztajnbok, None; E. Alexeeva, None; J. Antón, AbbVie, 2, Bristol-Myers Squibb, 2, Gebro, 2, GlaxoSmithKline, 2, Novartis, 2, Novimmune, 2, Pfizer, 2, Roche, 2, Sobi, 2; B. Feldman, Pfizer, 5, BMS, 5, Abbvie, 5, OPTUM, 5, AGILITY, 5; M. Katsicas, None; V. Smith, None; T. Avcin, None; R. Cimaz, None; M. Kostik, None; T. Lehman, None; W. Sifuentes-Giraldo, None; N. Vasquez-Canizares, None; S. Appenzeller, None; M. Janarthanan, None; M. Moll, None; D. Nemcova, None; M. Santos, AbbVie, 8, Biogen, 8, Novartis, 8, Pfizer, 8, Roche, 8; D. Schonenberg, None; C. Battagliotti, None; L. Berntson, None; B. Bica, None; J. Brunner, None; P. Costa Reis, None; D. Eleftheriou, None; L. Harel, None; G. Horneff, Chugai, 5, 8, GlaxoSmithKline, 5, 8, Novartis, 5, 8, Sanofi, 5, 8; T. Kallinich, None; D. Lazarevic, None; K. Minden, AbbVie, 8, Abbvie, 8, German Arthritis Foundation, 2, GlaxoSmithKline, 5, GSK, 5, Sanofi, 8; S. Nielsen, None; F. Nuruzzaman, None; A. Patwardhan, None; Y. Uziel, None; D. Kaiser, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Torok K, Stanevicha V, Terreri M, Sztajnbok F, Alexeeva E, Antón J, Feldman B, Katsicas M, Smith V, Avcin T, Cimaz R, Kostik M, Lehman T, Sifuentes-Giraldo W, Vasquez-Canizares N, Appenzeller S, Janarthanan M, Moll M, Nemcova D, Santos M, Schonenberg D, Battagliotti C, Berntson L, Bica B, Brunner J, Costa Reis P, Eleftheriou D, Harel L, Horneff G, Kallinich T, Lazarevic D, Minden K, Nielsen S, Nuruzzaman F, Patwardhan A, Uziel Y, Kaiser D, Helmus N. Is the Presentation and Severity Different of the Juvenile Diffuse and Limited Subtype Systemic Sclerosis? Results of Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/is-the-presentation-and-severity-different-of-the-juvenile-diffuse-and-limited-subtype-systemic-sclerosis-results-of-juvenile-scleroderma-inception-cohort/. Accessed .

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