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THU0229 Is There A Difference in The Presentation of Diffuse and Limited Subtype in Childhood? Results from The Juvenile Scleroderma Inception Cohort
  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. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc.

Objectives comparison of patients characteristic at the time of inclusion with ljSSc and djSSc

Methods We compered the demographics and clinical characteristics of the ljSSc and djSSc.

Results Up till now 74 patients were enrolled, 54 (76%) with djSSc and 18 with ljSSc (24%). 9% in djSSc and 25% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.3 years in ljSSc. 82% in the djSSc and 78% in the ljSSc group were female. The mean age of the onset of Raynaud symptomatic was 9.0 years in the jdSSc and 9.9 years in ljSSc group and onset of the non-Raynaud symptomatic with 9.4 in djSSc and 10.6 ljSSc. At the time of inclusion the mean modified Rodnan Skin Score was 18.5 in the djSSc and 8,4 in ljSSc (p=0.0001). Anti-Scl 70 positivity was found in 31.5% of djSSc and 30.8% in ljSSc. Only 2 patient in the djSSc group and one in the ljSSc group was anticentromere positive. Capillary changes occurred in 60.7% in the djSSc and 50% in ljSSc, but 58.2% in djSSc and only 23.5% in ljSSc had already history of ulcerations (p=0.013) and 21.8% had active ulceration in the djSSc and 5.9% in the ljSSc. 33.3% of djSSc and 72.7% of ljSSc had cardiac involvement (p=0.027). pulmonary hypertension occurred in 57.1% djSSc and 18.2% in ljSSc. 63% in djSSc and 27.3% in ljSSc group had signs of interstitial lung disease on imaging (p=0.046). Renal involvement occurred in 7.1% djSSc and 5.6% in ljSSc. 39.3% of djSSc and 22.2% of ljSSc had gastrointestinal involvement. 56.4% in djSSc and 83.35 in ljSSc had musculoskeletal involvement (p=0.04).

Conclusions Patients with djSSc have younger age at onset, have more often capillary changes and active ulcerations, pulmonary hypertension and less gastorintestinal and joint involvement and more disease damage. The characteristics of the pediatric subtypes differs from adults with SSc, especially the high proportion of patients with diffuse subtype.

Disclosure of Interest None declared

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