Is There a Difference in the Presentation of Diffuse and Limited Subtype in Childhood? Results from the Juvenile Scleroderma Inception Cohort

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Valda Stanevicha⁵, Maria Teresa Terreri⁶, 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 Helmus¹, ¹Hamburg Center for Pediatric and Adolescent Rheumatology, 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, ⁷Scientific Centre of Children’s Health of RAMS, Moscow, Russia, ⁸Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, ⁹Pediatric Rheumatology, Anna Meyer Children's Hospital, Florence, 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, ¹⁴Pediatric Rheumatology Division, Adolescent Health Care Unit, Universida de do Estado 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

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. Several adult publications looked at the differences between limited (ljSSc) and diffuse subtype (djSSc). There is few data regarding this topic in pediatric jSSc. The juvenile scleroderma inception cohort is a prospective standardized register for patients with jSSc.

Methods: Patients with jSSc were included worldwide to the jSSc cohort. Patients fulfilled the ACR criteria for jSSc. We compared the demographics and clinical features of the ljSSc and djSSc patients.

Results: Up till now 80 patients were enrolled, 58 (72.5%) with djSSc and 22 (27.5%) with ljSSc. 10% in djSSc and 23% in ljSSc showed overlap features. Disease duration at time of inclusion in the cohort was 3.7 years in the djSSc and 3.0 years in ljSSc. 83% in the djSSc and 81% in the ljSSc group were female. The mean age at onset of Raynaud symptoms was 9.0 years in the jdSSc and 10.4 years in ljSSc group and the mean age at onset of the non-Raynaud symptoms was 9.4 in djSSc and 10.9 ljSSc. At the time of inclusion the mean of modified Rodnan Skin Score was 18.2 in the djSSc and 9.1 in ljSSc. Anti-Scl 70 positivity was found in 30.4% of djSSc and 33.3% in ljSSc. Only 2 patients in the djSSc group and 2 in the ljSSc group presented anticentromere positive. Capillary changes occurred in 62.1% in the djSSc and 54.5% in ljSSc . History of active ulceration was present in 56% in the djSSc and in18% in ljSSc. Active ulcerations were present in 21.1% in the djSSc and 4.5% in the ljSSc. The mean of 6 Minute walk test was 392 m in the djSSc group and 504 m in the ljSSc group. 15.5% of djSSc and 41% of ljSSc had cardiac involvement. Pulmonary hypertension occurred in 8% in djSSc and 13% in ljSSc. 56% in djSSc and 31% in ljSSc group had signs of interstitial lung disease on imaging. Renal involvement occurred in 7% in djSSc and 4.5 % in ljSSc. None had systemic hypertension. 38% of djSSc and 18% of ljSSc had gastrointestinal involvement. 57% in djSSc and 73% in ljSSc had musculoskeletal involvement. Patient global disease activity measured by VAS was 44.2 in the djSSc and 46.3 in ljSSc. Patient global disease damage was 42.6 in the djSSc and 33.8 in ljSSc. Physician global disease activity measured by VAS was 40.3 in the djSSc and 25 in the ljSSc and physician global disease damage measured by VAS was 35.4 in the djSSc and 15.0 in the ljSSc.Mean CHAQ score was 0.4 in both groups.

Conclusion: Patients with djSSc were younger at onset; have more often capillary changes, active ulcerations and gastrointestinal involvement, and less pulmonary hypertension and musculoskeletal involvement. They also present moresevere disease and more disease damage. Features of the pediatric subtypes of jSSc differ from adults with SSc, especially the high proportion of patients with diffuse subtype.

Disclosure: I. Foeldvari, None; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, Pfzer, 2,AbbVie, Roche, 5; M. T. Terreri, 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, Terreri MT, 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. Is There a Difference in the Presentation of Diffuse and Limited Subtype in Childhood? Results from the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/is-there-a-difference-in-the-presentation-of-diffuse-and-limited-subtype-in-childhood-results-from-the-juvenile-scleroderma-inception-cohort/. Accessed .

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/is-there-a-difference-in-the-presentation-of-diffuse-and-limited-subtype-in-childhood-results-from-the-juvenile-scleroderma-inception-cohort/