Is There a Difference in the Presentation of Diffuse and Limited Subtype of Juvenile Systemic Sclerosis in Childhood? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com

Ivan Foeldvari¹, Jens Klotsche², Valda Stanevicha³, Maria M. Katsicas⁴, Maria Teresa Terreri⁵, Ana Paula Sakamoto⁶, Rolando Cimaz⁷, Mikhail Kostik⁸, Tadey Avcin⁹, Maria Jose Santos¹⁰, Monika Moll¹¹, Dana Nemkova¹², Flavio Sztajnbok¹³, Cristina Battagliotti¹⁴, Juergen Brunner¹⁵, Despina Eleftheriou¹⁶, Alberto Sifuentes Giraldo¹⁷, Liora Harel¹⁸, Mahesh Janarthanan¹⁹, Tilmann Kallinich²⁰, Kirsten Minden²¹, Susan Mary Nielsen²², Kathryn S. Torok²³, Yosef Uziel²⁴ and Nicola Helmus²⁵, ¹Hamburger Zentrum für Kinder-und Jugendrheumatologie, Hamburg, Germany, ²Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ³Pediatric cathedra, University Childrens Hospital, Riga, Latvia, ⁴Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, ⁵Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, ⁶Assistant doctor, Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ⁷Department of Paediatrics, University of Florence and Anna Meyer Children's Hospital, Florence, Italy, Florence, Italy, ⁸State Pediatric Medical University, Saint-Petersburg, Russia, ⁹University Children's Hospital, Ljubljana, Slovenia, ¹⁰Rheumatology, Hospital Garcia de Orta, Almada, Portugal, ¹¹Pediatric Rheumatology, University Childrenhospital, Tübingen, Germany, ¹²Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ¹³Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁴Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ¹⁵Universitätsklinik für Kinder- u. Jugendheilkunde, Innsbruck, Austria, ¹⁶Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, ¹⁷Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹⁸Pediatric Rheumatology Unit, Schneider Children's Medical Center,Tel Aviv University, Petach Tikvah, Israel, ¹⁹Pediatric Rheumatology, Chennai, India, ²⁰Charite, University Medicine Berlin, Berlin, Germany, ²¹Chidlrens´ hospital, Charité University Medicine, Berlin, Germany, ²²Rigshospitalet, Copenhagen, Denmark, ²³Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ²⁴Meir Medical Center, Kfar Saba, Israel, ²⁵Hamburg 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. Several publications in adults looked at the differences between limited and diffuse subtypes. There is rarity of data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort (www.juvenile-scleroderma.com) is a prospective standardized register for patients with jSSc.

Methods:

Patients with jSSc were included worldwide into the juvenile scleroderma inception cohort. We compared the demographics and clinical features of the ljSSc and djSSc. Data of the juvenile systemic sclerosis inception cohort have been contributed to the DeSScipher project which was funded by a grant of theEuropean Community’s Framework Programme 7 under grant agreement N° 305495.

Results:

Up till now 50 patients were enrolled, 34 (68%) with djSSc and 16 (32%) with ljSSc. 5 in the diffuse (15%) and 3 in the limited subtype (19%) had an overlap feature. The mean follow up of the patients in the cohort was 6.4 years in the djSSc and 5.5 years in ljSSc. 74% in the djSSc and 75% in the ljSSc group were female. The mean age at the onset of Raynaud´s Phenomenon was 8.4 years in the jdSSc and 10.8 years in ljSSc group (p=0.061) while the mean age at the onset of the first non-Raynaud presentation was 8.8 years in djSSc and 11.6 years ljSSc (p=0.010). At the time of the inclusion the mean modified Rodnan Skin Score was 20.4 in the djSSc and 8.9 in ljSSc (p=0.002). 72% in jdSSc and 56% in the ljSSc of patients (p=0.35) had already capillary changes, but 64% in djSSc and only 26% in ljSSc (p=0.015) had already history of ulcerations and 33% presented with active ulceration in the djSSc and 6% in the ljSSc (p=0.074). 71% of djSSc and 68 % of ljSSc had cardiopulmonary involvement. Four patients had pulmonary hypertension. 3 of them had djSSc. 44% in djSSc and 19% in ljSSc group (p=0.117) showed signs of interstitial lung disease on imaging. Four patients had renal involvement, 3 of them had djSSc, no renal crisis was reported. 47% in the djSSc and 25% in the ljSSc (p=0.216) had gastrointestinal involvement. Around 76% in djSSc and 81% in ljSSc had musculoskeletal involvement. Anti-Scl 70 positivity was found in 10/27 (37%) of djSSc and 5/13 (38%) in ljSSc. Only 2 patients and only in the djSSc group had anticentromere antibody.

Conclusion:

We present the data on the first 50 patients with jSSc included in our cohort. Patients with djSSc and ljSSc differ in several characteristics. Patients with djSSc were younger at onset, had more often capillary changes and active ulcerations, pulmonary hypertension and renal involvement. The characteristics of the pediatric subtypes differs from adults with SSc.

Disclosure: I. Foeldvari, None; J. Klotsche, None; V. Stanevicha, 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, Stanevicha V, 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. Is There a Difference in the Presentation of Diffuse and Limited Subtype of Juvenile Systemic Sclerosis in Childhood? Results from the Juvenile Scleroderma Inception Cohort Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/is-there-a-difference-in-the-presentation-of-diffuse-and-limited-subtype-of-juvenile-systemic-sclerosis-in-childhood-results-from-the-juvenile-scleroderma-inception-cohort-www-juvenile-scleroderma-co/. Accessed .

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