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THU0230 Is There A Difference in The Presentation of Male and Female Patients with Juvenile Systemic Sclerosis? 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 male and female patients with Systemic Sclerosis. There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (www.juvenilescleroderma.com) is a prospective standardized register for patients with jSSc.

Objectives comparison of patients characteristic at the time of inclusion in the registry who are male or female.

Methods Patients with jSSc were included worldwide to the juvenile scleroderma inception cohort. We compered the demographics and clinical characteristics of the male and female patients.

Results Up till now 74 patients were enrolled, 54 with djSSc (76%) and 18 with ljSSc (24%). 14 (19%) of the patients were male (M) and 60 female (F) (81%). The mean disease duration at the time of inclusion was 9.3 in M and 9.2 in F patients. 74.4% of the M and 76.7% of the F had diffuse subset. The mean age of the onset of Raynaud symptomatic was 9.3 in M and 9.2 years in the F patients and the non-Raynaud symptomatic with 9.1 in M and 9.9 in F patients. At the time of the inclusion the mean modified Rodnan Skin Score was 20 in M and 15.1 in F patients. Anti-Scl 70 positivity was found in 42.9% in M and 32.1% in F patients. Anticentromere positivity occurred in 16.7% in M and 3.3% in F patients (p=0.027). Capillary changes were present in 50% of the M and 60% of F patients, but 50% in M and F had already history of ulcerations, but 28.6% in M and 15.5% in F had active ulceration. 57.1% of the M and 50% of the F patients had cardiopulmonary involvement. Six patients had pulmonary hypertension, they were all F. 75% of M and 46.7% of F patients had signs of interstitial lung disease on imaging. Renal involvement was around 7% in both sexes. 21.4% in M and 38.3% in F patients had gastrointestinal involvement. 92.9% of M and 55.9% in M patients had musculoskeletal involvement.

Conclusions We present the data on the first 74 patients with jSSc included in our cohort. Patients with male sex have a more severe disease similar to adult male patients.

Disclosure of Interest None declared

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