Performance of Juvenile Scleroderma Classification Criteria for Juvenile Systemic Sclerosis. Results from the Jssc Inception Cohort

Jens Klotsche¹, Ivan Foeldvari², Ozgur Kasapcopur³, Vanessa Smith⁴, Flavio Sztajnbok⁵, Maria M. Katsicas⁶, Rolando Cimaz⁷, Mahesh Janarthanan⁸, Jordi Anton⁹, Mikhail Kostik¹⁰, Dana Nemkova¹¹, Walter A. Sifuentes-Giraldo¹², Valda Stanevicha¹³, Kathryn S. Torok¹⁴, Simone Appenzeller¹⁵, Tadey Avcin¹⁶, Lillemor Berntson¹⁷, Liora Harel¹⁸, Tilmann Kallinich¹⁹, Maria José Santos²⁰, Maria Teresa Terreri²¹, Yosef Uziel²² and Nicola Helmus², ¹Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ²Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ³Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, ⁴Faculty of Internal Medicine, Ghent University, Ghent, Belgium, ⁵Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ⁶Service of Immunology & Rheumatology., Hospital de Pediatría Prof Dr JP Garrahan, Buenos Aires, Argentina, ⁷Pediatrics, Ospedale Pediatrico Anna Meyer, Florence, Italy, ⁸Pediatric Rheumatology, Chennai, India, ⁹pediatric Rheumatology, University Childrenxs Hospital, Barcelona, Spain, ¹⁰Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ¹¹Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ¹²Rheumatology, Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹³Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ¹⁴Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, ¹⁵Pediatric Rheumatology Unit, State University of Campinas, Campinas, Brazil, ¹⁶University Children's Hospital, Ljubljana, Slovenia, ¹⁷Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden, ¹⁸Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, ¹⁹Charité, Humbolt University Medicine Berlin, Berlin, Germany, ²⁰Reuma.pt, Almada, Portugal, Almada, Portugal, ²¹Pediatric Rheumatology Unit, Federal University of São Paulo (UNIFESP - Universidade Federal de São Paulo), São Paulo, Brazil, ²²Meir Medical Center, Kfar Saba, Israel

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:

Juvenile systemic sclerosis (jSSc) is a rare disease during childhood. Classification criteria for jSSc were published in 2007. They include a major criterion that is required and 20 minor criteria from which at least two are required for classification. The objective of this study was to investigate the distribution of the single minor classification criteria in patients with jSSc in relation to sex and the age at disease onset in a prospective cohort study.

Methods:

Data from the prospective international inceptions cohort for jSSc was used to investigate the proportion of jSSc patients fulfilling the 20 minor criteria. 48 patients with a jSSc disease duration of less than 2.5 years were included in the analyses. Age at disease onset (AO) was categorized into two groups (<12 (AO G1) /≥ 12 (AO G2) years). Latent class analysis (LCA) was used to identify distinct groups of patients which share a similar pattern of minor criteria for jSSc.

Results:

The mean physician global on a visual analogue scale was 46 (sd=26) at enrolment, 37 (77.1%) were female, and 31 (64.6%) patients had diffuse subtype. Raynaud’s phenomenon (90%), ANA positivity (87%), and nailfold capillary abnormalities (77%) were the most present minor criteria. Patients in group AO G1 met more often only 2 or 3 minor criteria (23% versus 11%), whereas almost 40% in AO G2 (versus 23% in AO G1) met more than 5 criteria. Patients in AO G2 had more often sclerodactyly (83%/47%), decreased DLCO (62%/38%) and were more often ANA positive (100%/72%) than in AO G1. LCA in patients of AO G2 resulted in two groups. In the first group, the leading minor criteria were sclerodactyly, Raynaud’s phenomenon, nailfold capillary abnormalities and SCL-70 positivity. The second group was characterized by patients with nailfold capillary abnormalities, Raynaud’s phenomenon, decreased DLCO, reflux and arthritis. Female patients fulfilled in mean significantly more minor criteria than boys (4.3 versus 5.1). Differences were highest in nailfold capillary abnormalities (84%/55%), gastro-esophageal reflux (30%/0%), and arthritis (35%/9%). No case with dysphagia, renal crisis, atrial hypertension, neuropathy and carpal tunnel syndrome was documented at enrolment.

Conclusion:

Patients with a jSSc disease onset before the age of 12 and males met significantly less minor criteria than patients with a later onset and females. We could recognize 2 patterns of presentation of jSSc over the age at onset of 12, which was not described before. We have to observe in the long term follow up, how fare to be part of a cluster present a prognostic factor for morbidity and mortality.

Disclosure: J. Klotsche, None; I. Foeldvari, None; O. Kasapcopur, None; V. Smith, None; F. Sztajnbok, None; M. M. Katsicas, None; R. Cimaz, None; M. Janarthanan, None; J. Anton, None; M. Kostik, None; D. Nemkova, None; W. A. Sifuentes-Giraldo, None; V. Stanevicha, None; K. S. Torok, None; S. Appenzeller, None; T. Avcin, None; L. Berntson, None; L. Harel, None; T. Kallinich, None; M. J. Santos, None; M. T. Terreri, None; Y. Uziel, None; N. Helmus, None.

To cite this abstract in AMA style:

Klotsche J, Foeldvari I, Kasapcopur O, Smith V, Sztajnbok F, Katsicas MM, Cimaz R, Janarthanan M, Anton J, Kostik M, Nemkova D, Sifuentes-Giraldo WA, Stanevicha V, Torok KS, Appenzeller S, Avcin T, Berntson L, Harel L, Kallinich T, Santos MJ, Terreri MT, Uziel Y, Helmus N. Performance of Juvenile Scleroderma Classification Criteria for Juvenile Systemic Sclerosis. Results from the Jssc Inception Cohort [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/performance-of-juvenile-scleroderma-classification-criteria-for-juvenile-systemic-sclerosis-results-from-the-jssc-inception-cohort/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/performance-of-juvenile-scleroderma-classification-criteria-for-juvenile-systemic-sclerosis-results-from-the-jssc-inception-cohort/