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46. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 32. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

19.09. - 22.09.2018, Mannheim

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Do Raynaud phenomenon negative juvenile systemic scleroderma patients have a different pattern of organ involvement as Raynaud phenomenon positive patients?

Meeting Abstract

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  • Ivan Foeldvari - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg
  • Jens Klotsche - Deutsches Rheuma-Forschungszentrum (DRFZ), Programmbereich Epidemiologie, Berlin
  • Ozgur Kasapcopur - Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
  • Amra Adrovic - Department of Pediatric Nephrology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
  • Kathryn Torok - University Childrens Hospital, Pittsburgh, USA
  • Valda Stanevicha - University Childrens Hospital, Riga, Latvia
  • M. T. Terreri - Universidade Federal de São Paulo, Pediatric Rheumatology, Sao Paulo, Brasil
  • Ekaterina Alexeeva - Russian Academy of Medical Sciences, Rheumatology Department, Scientific Center for Children’s Health, Moskau, Russland
  • Maria Katsicas - Hospital de Pediatria, Buenos Aires, Argentina
  • Vanessa Smith - Gent University Hospital, Rheumatology, Gent, Belgium
  • Flavio Sztajnbok - Hospital Universitario Pedro Ernesto, Rio de Janeiro, Brazil
  • Tadey Avcin - University Childrens Hospital, Pediatric Rheumatology, Ljubljana, Slovenia
  • Rolando Cimaz - University of Florence, Florence, Italy
  • Jordi Anton - University Children’s Hospital, Pediatric Rheumatology, Barcelona, Spain
  • Mikhail Kostik - Saint-Petersburg State Pediatric Medical University, St. Petersburg, Russia
  • Thomas Lehman - Hospital for Special Surgery, New York, USA
  • W. Alberto Sifuentes-Giraldo - University Hospital Ramón y Cajal, Madrid, Spain
  • Simone Appenzeller - Faculty of Medical Science State University of Campinas (UNICAMP), Sao Paulo, Brazil
  • Mahesh Janarthanan - Pediatric Rheumatology, Chennai, India
  • Monika Moll - Universitätsklinikum Tübingen, Klinik für Kinder- und Jugendmedizin, Tübingen
  • Dana Nemcova - University Childrens Hospital, Pediatric Rheumatology, Prague, Czech Republic
  • Maria Jose Santos - Serviço de Reumatologia, Hospital Garcia de Orta, Almada, Portugal
  • Cristina Battagliotti - Hospital den Ninos Dr. Orlando Alassia, Santa Fe, Argentina
  • Lillemor Berntson - Department of Pediatrics, Uppsala University Hospital, Uppsala, Sweden
  • Jürgen Brunner - Medizinische Universität Innsbruck, Department für Kinder- und Jugendheilkunde, Innsbruck, Österreich
  • Patrícia Costa Reis - Hospital de Santa Maria, Lisbon, Portugal
  • Despina Eleftheriou - Great Ormond Street Childrens Hospital, London, United Kingdom
  • Liora Harel - Pediatric Rheumatology, Nettnja, Israel
  • Tilmann Kallinich - Charité – Universitätsmedizin Berlin, Klinik für Pädiatrie mit Schwerpunkt Pneumologie und Immunologie, Sektion Rheumatologie, Berlin
  • Kirsten Minden - Deutsches Rheuma-Forschungszentrum (DRFZ) und Charité Universitätsmedizin Berlin, Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Berlin
  • Susan Nielsen - Juliane Marie Centret, Rigshospitalet, Pediatric Rheumatology, Copenhagen, Danmark
  • Yosef Uziel - Pediatric Rheumatology, Sapir Medical Center, Kfar Saba, Israel
  • Ann Stevens - Seattle Children’s, Seattle, USA
  • Clarissa Pilkington - Great Ormond Street Hospital, London, Vereinigtes Königreich
  • Nicola Helmus - Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg

Deutsche Gesellschaft für Rheumatologie. Deutsche Gesellschaft für Orthopädische Rheumatologie. Gesellschaft für Kinder- und Jugendrheumatologie. 46. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 32. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR). Mannheim, 19.-22.09.2018. Düsseldorf: German Medical Science GMS Publishing House; 2019. DocKR.03

doi: 10.3205/18dgrh117, urn:nbn:de:0183-18dgrh1171

Published: February 5, 2019

© 2019 Foeldvari et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Background: Juvenile systemic scleroderma (jSSc) is an orphan disease, with an estimated prevalence of 3 per 1000 000 children. Most jSSc patients primarily present with Raynaud phenomenon (RP). We investigated in our patient of the juvenile scleroderma inception cohort, how fare patients with (RP+) and without (RP-) RP differed in their clinical presentation at enrolment.

Methods: The jSSc is a prospective cohort of jSSc patients. Patients were enrolled who were diagnosed with jSSc, had a jSSc onset age under 16 years and were younger as age of 18 years at the time of inclusion. The patients are prospectively assessed every 6 months according to a standardized protocol. We reviewed the organ involvement pattern of our patients currently followed in the cohort.

Results: 100 patients are currently followed in the cohort and 89 (89%) of them had RP. The female/male ratio was lower in the RP+ group, 3.7:1 compared to 4.5:1(p=0.808). Diffuse subtype was more common in the RP+ group, 72% compared to 63%. Mean age of onset of first non- Raynaud symptomatic was 10.4 years in both groups. Mean disease duration was slightly higher in the RP+ group, 3.4 compared to 2.2 years. ANA positivity was higher in the RP+ group, 88% compared to 70% (p=0.48). Anti-Scl70 was 34% in the RP+ and 20% in the RP-group (p=0.34). Interestingly 7% of RP + but none of the RP+ were anti-centromere positive. The mean modified skin score was lower in RP + group (mean of 14.8 compared to 17.0). There were significantly more nailfold capillary changes (70% compared to 18%, p=0.001) and a higher rate of history of ulceration in the RP+ group (49% compared to 20%, p=0.083). Decreased DLCO and FVC<80% was higher in the RP-negative group with 45%/50% compared to 37.5%/31% respectively. Pulmonary hypertension occurred in 7% in the RP+ group and there was no case in the RP- group (p=0.335). RP- group had a higher rate of urinary sediment changes 18% compared to 4.5% in the RP+ group (p=0.07). No renal crisis or hypertension was reported in neither groups. Gastrointestinal involvement was similar between the two groups with around 35%. Occurrence of swollen joints was similar in both groups as the frequency of muscle weakness with around 20%. The tendon friction rub occurred around 10% in both groups. In the patient related outcomes, there was only a difference in rating of Raynauds activity.

Conclusion: The RP- group differed from RP + group in the clinical presentation at enrolment. The absence of Raynaud phenomenon was associated with a decreased rate of history of ulceration, no occurrence of pulmonary hypertension. Interestingly higher rate of urinary sedimentary changes and no anticentromere positivity was observed in RP- patients.