Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 80 Patients at First Assessment

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Valda Stanevicha⁵, Ana Paula Sakamoto⁶, Ekaterina Alexeeva⁷, Maria M. Katsicas⁸, Rolando Cimaz⁹, Mikhail Kostik¹⁰, Thomas J. A. Lehman¹¹, Walter A. Sifuentes-Giraldo¹², Vanessa Smith¹³, Flavio Sztajnbok¹⁴, Tadey Avcin¹⁵, Maria Jose Santos¹⁶, Dana Nemkova¹⁷, Cristina Battagliotti¹⁸, Despina Eleftheriou¹⁹, Liora Harel²⁰, Mahesh Janarthanan²¹, Tilmann Kallinich²², Jordi Anton²³, Kirsten Minden², Susan Mary Nielsen²⁴, Kathryn S. Torok²⁵, Yosef Uziel²⁶ and Nicola Helmus²⁷, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ³Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, ⁴Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, ⁵University Childrens Hospital, Riga, Latvia, ⁶Pediatric Rheumatology Unit, Federal University of São Paulo, São Paulo, Brazil, ⁷Rheumatology, Scientific Center of Children's Health, Moscow, Russia, ⁸Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, ⁹Pediatrics, University of Firenze, Firenze, Italy, ¹⁰Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ¹¹Chief Div Ped Rheum PTD, Hospital for Special Surgery, New York, NY, ¹²Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹³Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium, ¹⁴Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁵University Children's Hospital, Ljubljana, Slovenia, ¹⁶Rheumatology, Department of Rheumatology, Hospital Garcia de Orta, Almada, Portugal, Almada, Portugal, ¹⁷Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ¹⁸Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ¹⁹Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, ²⁰Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikvah, Israel, ²¹Pediatric Rheumatology, Chennai, India, ²²Charite, University Medicine Berlin, Berlin, Germany, ²³Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, ²⁴Rigshospitalet, Copenhagen, Denmark, ²⁵Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, PA, ²⁶Meir Medical Center, Kfar Saba, Israel, ²⁷Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: juvenile sclerosis and systemic sclerosis

Session Information

Date: Tuesday, November 15, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster III: Systemic JIA, Autoinflammatory Syndromes, Scleroderma, Vasculitis, Miscellaneous

Session Type: ACR Poster Session C

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

Background/Purpose: 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 project, where prospectively and with a standardized assessment data of jSSc patients are collected. We present the data of the patients at the entry into the cohort.

Methods: Patients with jSSc, according the ACR criteria, 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 80 patients were enrolled. Sixty-six (82.5%) of the 80 patients were female. The mean age of the onset of Raynaud symptomatic was 9.4 years (0.2 – 15.9). The mean age at the onset of the non-Raynaud symptomatic were 9,9 years (0.3 -15.9). 58 (72.5%) had diffuse subtype and 22 had limited subtype (27.5%). 11 (14%) of them have an overlap symptomatic, in 6 (10%) in the diffuse and 5 (23%) in limited subtype. At the time of the inclusion the mean modified Rodnan Skin Score was 15.7 (0-51). ANA positive were 60/77 (78%), 24/77 (31%) of them were anti-Scl 70 positive and 4/46 (9%) were anticentromere positive. 48/80 (60%) had already capillary changes and 36/78 (46%) inactive ulcerations, 13/78 (17%) had active ulceration at the time of the inclusion. 39/80 (49%) had cardiopulmonary involvement, 22/45 (49%) of had signs of interstitial lung disease on imaging, 16/36 (44%) had FVC <80% and 10/19 (52%) had DLCO < 80%. 6/44 (14%) patients had pulmonary hypertension. The mean 6 Minute Walk Test was 419.3 m (60-615). 5/80 (6%) had renal involvement, none of them had hypertension. 26/80 (32.5%) had gastrointestinal involvement, and 18/26 (69%) of them esophageal involvement. 48/79 (62%) had musculoskeletal involvement, mostly limited range of joint movement in 21/49 (43%). Isolated muscle weakness occurred in 3/46 (6.5%). 2/80 (2.5%) showed neurologic involvement. The mean CHAQ score (n=51) was 0.4 (0-2.5). Patient global disease activity (n=40) on VAS (0-100) was 44.4 and disease damage 41.6. Physician global of disease activity (n=44) on VAS (0-100) was 37.5 and physician global of disease damage (n=43) was 32.1.

Conclusion: We present the data on the first 80 patients with jSSc at the time of inclusion in our cohort. 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 72.5%. 14% of the patients had an overlap features. The patients had significant disease burden assessed by patients and physician at the entry of the cohort.

Disclosure: I. Foeldvari, None; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, Pfzer, 2,AbbVie, Roche, 5; A. P. Sakamoto, None; E. Alexeeva, None; M. M. Katsicas, None; R. Cimaz, None; M. Kostik, None; T. J. A. Lehman, None; W. A. Sifuentes-Giraldo, None; V. Smith, None; F. Sztajnbok, None; T. Avcin, None; M. J. Santos, None; D. Nemkova, None; C. Battagliotti, None; D. Eleftheriou, None; L. Harel, None; M. Janarthanan, None; T. Kallinich, None; J. Anton, None; K. Minden, None; S. M. Nielsen, None; K. S. Torok, None; Y. Uziel, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Sakamoto AP, Alexeeva E, Katsicas MM, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Smith V, Sztajnbok F, Avcin T, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Janarthanan M, Kallinich T, Anton J, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 80 Patients at First Assessment [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/update-on-the-juvenile-systemic-sclerosis-inception-cohort-project-characteristics-of-the-first-80-patients-at-first-assessment/. Accessed .

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