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SAT0257 Update on The Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of The First 74 Patients at First Assessment
  1. I. Foeldvari1,
  2. J. Klotsche2,
  3. O. Kasapcopur3,
  4. A. Adrovic3,
  5. M.T. Terreri4,
  6. V. Stanevicha5,
  7. M.M. Katsikas6,
  8. E. Alexeeva7,
  9. F. Sztajnbok8,
  10. R. Cimaz9,
  11. M. Kostik10,
  12. W.A. Sifuentes-Giraldo11,
  13. T. Lehman12,
  14. D. Nemcova13,
  15. M. Moll14,
  16. M.J. Santos15,
  17. T. Avcin16,
  18. C. Battagliotti17,
  19. J. Brunner18,
  20. S. Nielsen19,
  21. T. Kallinich2,
  22. K. Minden2,
  23. M. Janarthanan20,
  24. L. Harel21,
  25. Y. Uziel22,
  26. D. Eleftheriou23,
  27. K.S. Torok24,
  28. N. Helmus1
  1. 1Hamburg Center for Pediatric and Adolescent Rheumatology, Am Schön Klinik Eilbek, Hamburg
  2. 2Uni Child Hosp, Berlin, Germany
  3. 3Uni Child Hosp, Istanbul, Turkey
  4. 4Uni Child Hosp, São Paulo, Brazil
  5. 5Uni Child Hosp, Riga, Latvia
  6. 6Uni Child Hosp, Buenos Aires, Argentina
  7. 7Uni Child Hosp, Moscow, Russian Federation
  8. 8Uni Child Hosp, Rio de Janeiro, Brazil
  9. 9Uni Child Hosp, Firenze, Italy
  10. 10Uni Child Hosp, Saint-Petersburg, Russian Federation
  11. 11Uni Child Hosp, Madrid, Spain
  12. 12Uni Child Hosp, New York, United States
  13. 13Uni Child Hosp, Prague, Czech Republic
  14. 14Uni Child Hosp, Tuebingen, Germany
  15. 15Uni Child Hosp, Almada, Portugal
  16. 16Uni Child Hosp, Ljubljana, Slovenia
  17. 17Uni Child Hosp, Santa Fe, Argentina
  18. 18Uni Child Hosp, Innsbruck, Austria
  19. 19Uni Child Hosp, Copenhagen, Denmark
  20. 20Uni Child Hosp, Chennai, India
  21. 21Uni Child Hosp, Tel Aviv
  22. 22Uni Child Hosp, Kfar Saba, Israel
  23. 23Uni Child Hosp, London, United Kingdom
  24. 24Uni Child Hosp, Pittsburgh, United States

Abstract

Background Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently just retrospective data exist regarding evolvement of organ involvement. In the retrospective studies assessment of the organ involvement is not standardized. Our project is the first one, where prospectively and with a standardized assessment data of jSSc patients are collected.

Objectives to learn about the characteristics and evolvement of jSSc

Methods Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol.

Results 26 centers from 17 countries applied to participate on the project. The assent and consent forms were translated into the local native languages. Up till now 74 patients were enrolled. Sixty (81%) of the 74 patients were female. The mean age of the onset of Raynaud symptomatic was 9.2 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,7 years (0.3 -15.9). 56 (76%) of the 74 have diffuse subtype, 10 (14%) of them have an overlap symptomatic. At the time of the inclusion the mean modified Rodnan Skin Score was 16.0. ANA positive were 55/71 (77%), 24/70 (34%) of them were anti-Scl 70 positive and 3/42 (7%) was anticentromere positive. 43/74 (58%) had already capillary changes and 36/72 (50%) inactive ulcerations, 13/72 (18%) had active ulceration at the time of the inclusion. 38/74 (51%) had cardiopulmonary involvement, 19/38 (50%) of had signs of interstitial lung disease on imaging, 18/42 (43%) had FVC <80% and 12/21 (57%) had DLCO <80%. 6/38 (16%) patients had pulmonary hypertension. 5/74 (7%) had renal involvement. 26/74 (35%) had gastrointestinal involvement, and 23/26 (88%) of them esophageal involvement. 46/73 (63%) had musculoskeletal involvement. 2/74 (3%) showed neurologic involvement. The mean CHAQ score was 0.4 (0–2.5). Patient global disease activity on VAS (0–100) was 44.9 and disease damage 41.6. Physician global of disease activity on VAS (0–100) was 39.7 and physician global of disease damage was 34.6.

Conclusions The current recruitment data confirms that pediatric patients are different from the adult patients, there is a significantly higher proportion of diffuse subset patients with 81%. 14% of the patients have overlap features.

Disclosure of Interest None declared

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