Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 50 Patients at First Assessment. Www.Juvenile-Scleroderma.Com

Ivan Foeldvari¹, Jens Klotsche², Valda Staņeviča³, Ekaterina Alexeeva⁴, Maria M. Katsicas⁵, Maria Teresa Terreri⁶, Ana Paula Sakamoto⁷, Rolando Cimaz⁸, Mikhail Kostik⁹, Tadey Avcin¹⁰, Maria Jose Santos¹¹, Monika Moll¹², Dana Nemkova¹³, Flavio Sztajnbok¹⁴, Cristina Battagliotti¹⁵, Juergen Brunner¹⁶, Despina Eleftheriou¹⁷, Alberto Sifuentes Giraldo¹⁸, Liora Harel¹⁹, Mahesh Janarthanan²⁰, Tilmann Kallinich²¹, Kirsten Minden²², Susan Mary Nielsen²³, Kathryn S. Torok²⁴, Yosef Uziel²⁵ and Nicola Helmus²⁶, ¹Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, ²Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ³Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ⁴Rheumatology, Scientific Center of Children's Health, Moscow, Russia, ⁵Immunology & Rheumatology., Hospital de Pediatrìa Garrahan, Buenos Aires, Argentina, ⁶Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, ⁷Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ⁸Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, ⁹State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁰University Children's Hospital, Ljubljana, Slovenia, ¹¹Rheumatology, Hospital Garcia de Orta, Almada, Portugal, ¹²Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, ¹³Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ¹⁴Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁵Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ¹⁶Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, ¹⁷Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, ¹⁸Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹⁹Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, ²⁰Pediatric Rheumatology, Chennai, India, ²¹Charite, University Medicine Berlin, Berlin, Germany, ²²Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, ²³Rigshospitalet, Copenhagen, Denmark, ²⁴Pediatric Rheumatology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ²⁵Meir Medical Center, Kfar Saba, Israel, ²⁶Hamburg Centre for Pediatric and Adolescent Rheumatology, Hamburg, Germany

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Systemic sclerosis

Session Information

Date: Sunday, November 8, 2015

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster I: Lupus, Scleroderma, JDMS

Session Type: ACR Poster Session A

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 previous retrospective studies assessment of the organ involvement was not standardized. Our project is the first one, where data of jSSc patientes were collected prospectively and with a standardized assessment.

Methods: Patients with jSSc were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol. Data of the juvenile systemic sclerosis inception cohort have been contributed to the DeSScipher project which was funded by a grant of the European Community’s Framework Programme 7 under grant agreement N° 305495.”

Results:

24 centers from 16 countries agreed to participate on the project. The assent and consent forms were translated into the local native languages. Until now 50 patients have been enrolled with a mean disease duration of 6.1 years. Thirtyseven (74%) of the 50 patients were females. The mean age of the onset of Raynaud´s phenomenon was 9.2 years (2-16 years), the youngest 2 years old. The mean age at the onset of the non-Raynaud presentation of jSSc was 9.7 years (2.3-16.00 years). 34 (68%) of the 50 have diffuse subtype. 5 in the diffuse (14.7%) and 3 in the limited subtype (19%) had an overlap feature.

At the time of the inclusion the mean modified Rodnan Skin Score was 16.7. 32/48 (67%) had already capillary changes and 25/48 (52%) already had history of ulcerations, 12/48 (25%) had active ulcerations at the time of the inclusion. 35/50 (70%) had cardiopulmonary involvement, 18/50 (36%) had signs of interstitial lung disease. 5 (10%) patients had pulmonary hypertension. Four (8%) had renal involvement, but no renal crisis. 20/50 (40%) had gastrointestinal involvement and 15 (30%) of them esophageal involvement. 38/49 (77.5%) had musculoskeletal involvement. ANA positivity occurred in 36/47 (77%) and 15/40 (37.5%) of them were anti-Scl 70 positive. 2/29 (7%) had anticentromere positivity.

Conclusion: We present the data on the first 50 patients with jSSc included in our cohort. The current recruitment data confirms that pediatric patients are different from the adult patients, with a higher proportion of diffuse subset patients with 68% and 16% of patients with overlap features. Anti-centromere antibodies occuring only in 7% of patients.

Disclosure: I. Foeldvari, None; J. Klotsche, None; V. Staņeviča, None; E. Alexeeva, None; M. M. Katsicas, None; M. T. Terreri, None; A. P. Sakamoto, None; R. Cimaz, None; M. Kostik, None; T. Avcin, None; M. J. Santos, None; M. Moll, None; D. Nemkova, None; F. Sztajnbok, None; C. Battagliotti, None; J. Brunner, None; D. Eleftheriou, None; A. Sifuentes Giraldo, None; L. Harel, None; M. Janarthanan, None; T. Kallinich, 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, Staņeviča V, Alexeeva E, Katsicas MM, Terreri MT, Sakamoto AP, Cimaz R, Kostik M, Avcin T, Santos MJ, Moll M, Nemkova D, Sztajnbok F, Battagliotti C, Brunner J, Eleftheriou D, Sifuentes Giraldo A, Harel L, Janarthanan M, Kallinich T, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Update on the Juvenile Systemic Sclerosis Inception Cohort Project. Characteristics of the First 50 Patients at First Assessment. Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/update-on-the-juvenile-systemic-sclerosis-inception-cohort-project-characteristics-of-the-first-50-patients-at-first-assessment-www-juvenile-scleroderma-com/. Accessed .

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