Male Patients Have a More Severe Course As Female Patients with Diffuse Juvenile Systemic Scleroderma? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Valda Stanevicha⁵, Maria Teresa Terreri⁶, Ekaterina Alexeeva⁷, Maria M. Katsicas⁸, Vanessa Smith⁹, Rolando Cimaz¹⁰, Mikhail Kostik¹¹, Thomas J. A. Lehman¹², Jordi Anton¹³, Walter A. Sifuentes-Giraldo¹⁴, Flavio Sztajnbok¹⁵, Tadey Avcin¹⁶, Mahesh Janarthanan¹⁷, Maria José Santos¹⁸, Dana Nemkova¹⁹, Cristina Battagliotti²⁰, Despina Eleftheriou²¹, Liora Harel²², Tilmann Kallinich²³, K Minden²⁴, Susan Mary Nielsen²⁵, Kathryn S. Torok²⁶, Yosef Uziel²⁷, Anne Stevens²⁸, Clarissa Pilkington²⁹ and Nicola Helmus¹, ¹Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ²Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ³Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, ⁴Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, ⁵Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ⁶Pediatric Rheumatology Unit, Federal University of São Paulo (UNIFESP - Universidade Federal de São Paulo), São Paulo, Brazil, ⁷Children's Health of RAMS and IM Sechenov First Moscow State Medical University, Moscow, Russian Federation, ⁸Service of Immunology & Rheumatology., Hospital de Pediatría Prof Dr JP Garrahan, Buenos Aires, Argentina, ⁹Faculty of Internal Medicine, Ghent University, Ghent, Belgium, ¹⁰Pediatrics, Ospedale Pediatrico Anna Meyer, Florence, Italy, ¹¹Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ¹²Pediatric Rheumatology, Hospital for Special Surgery Weill Cornell Medical College, New York, NY, ¹³pediatric Rheumatology, University Childrenxs Hospital, Barcelona, Spain, ¹⁴Rheumatology, Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁵Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁶University Children's Hospital, Ljubljana, Slovenia, ¹⁷Pediatric Rheumatology, Chennai, India, ¹⁸Reuma.pt, Almada, Portugal, Almada, Portugal, ¹⁹Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ²⁰Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ²¹Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, ²²Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, ²³Charité, Humbolt University Medicine Berlin, Berlin, Germany, ²⁴Charité – University of Medicine Berlin, Berlin, Germany, ²⁵Rigshospitalet, Copenhagen, Denmark, ²⁶Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, ²⁷Meir Medical Center, Kfar Saba, Israel, ²⁸University of Washington, Department of Pediatrics, Seattle, WA, ²⁹Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Juvenile scleroderma and pediatric rheumatology

Session Information

Date: Monday, November 6, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster II: Lupus and Related Disorders, Myositis, Scleroderma and Vasculitis

Session Type: ACR Poster Session B

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

Background/Purpose:

In adult systemic scleroderma patients male have a more sever course. This issue was never evaluated in a larger juvenile scleroderma cohort.

Methods:

Patients with jSSc were included worldwide to the jSScI cohort. We compared the demographics and clinical characteristics of male and female dcjSSc patients at the time of the enrollment into the jSSci.

Results:

Sixty-two dcjSSc patients were enrolled Up till April 2016. 11/62 (18%) of the patients were male (M) and 51/62 female (F) (82%). The mean age of the onset of Raynaud symptoms was 8.0 in M and 9.4 years in the F patients and the non-Raynaud symptoms was 8.2 in M and 10.0 in F patients. At the time of the enrollment the mean MRSS was 24.3 in M and 17.3 in F patients. Anti-Scl 70 positivity was found in 4/11 (36.4%) in M and 14/49 (28.6%) in F patients. Anticentromere positivity occurred in 2/11 (18.2%) in M and 0/23 (0%) in F patients (p=0.035). Capillary changes were present in 8/11(73%) of the M and 30/51 (59%) of F patients, but 36% of M and F had already history of ulcerations. 7/11 (64%) of the M and 21/51 (41%) of the F patients had cardiopulmonary involvement. pulmonary hypertension occurred in F patients (n=6). 7/11 (64%) of M and 11/51 (22%) of F patients had signs of interstitial lung disease (p=0.005). No renal crisis was observed till enrollment. 37% in both sexes had gastrointestinal involvement. Patient global disease damage was on a VAS (0- 100) 56.9 in M and in 38.4 in F (p=0.014) and patient global disease activity was 58.8 in M and 41.9 in M (p=0.024). Physician global of disease activity on a VAS was 58.9 in M and 36.9 in F (p=0.004) and physician global disease damage was 60.2 in M and 31.2 in F (p=0.001).

Conclusion:

Male patients had a significantly more severe disease course at time point of enrollment . This finding overlaps to the observation in adult cohorts.

Disclosure: I. Foeldvari, None; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, None; M. T. Terreri, None; E. Alexeeva, None; M. M. Katsicas, None; V. Smith, None; R. Cimaz, None; M. Kostik, None; T. J. A. Lehman, None; J. Anton, None; W. A. Sifuentes-Giraldo, None; F. Sztajnbok, None; T. Avcin, None; M. Janarthanan, None; M. J. Santos, None; D. Nemkova, None; C. Battagliotti, None; D. Eleftheriou, None; L. Harel, None; T. Kallinich, None; K. Minden, None; S. M. Nielsen, None; K. S. Torok, None; Y. Uziel, None; A. Stevens, None; C. Pilkington, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Terreri MT, Alexeeva E, Katsicas MM, Smith V, Cimaz R, Kostik M, Lehman TJA, Anton J, Sifuentes-Giraldo WA, Sztajnbok F, Avcin T, Janarthanan M, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Kallinich T, Minden K, Nielsen SM, Torok KS, Uziel Y, Stevens A, Pilkington C, Helmus N. Male Patients Have a More Severe Course As Female Patients with Diffuse Juvenile Systemic Scleroderma? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/male-patients-have-a-more-severe-course-as-female-patients-with-diffuse-juvenile-systemic-scleroderma-results-from-the-juvenile-scleroderma-inception-cohort-www-juvenile-scleroderma-com/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/male-patients-have-a-more-severe-course-as-female-patients-with-diffuse-juvenile-systemic-scleroderma-results-from-the-juvenile-scleroderma-inception-cohort-www-juvenile-scleroderma-com/