Performance of the Adult Systemic Sclerosis Classification in Juvenile Systemic Sclerosis Patients. Results from the Juvenile Systemic Sclerosis Inception Cohorte Www.Juvenile-Scleroderma.Com

Ivan Foeldvari¹, Jens Klotsche², Valda Stanevicha³, 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, ³University Childrens Hospital, Riga, Latvia, ⁴Rheumatology, Scientific Center of Children's Health, Moscow, Russia, ⁵Hospital de Pediatria, 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, ⁸Pediatric Rheumatology, Anna Meyer Children's Hospital, 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, ¹⁴Pediatric Rheumatology Division, Adolescent Health Care Unit, Universida de do Estado 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, ²²Epidemiology, Charite, DRFZ, Berlin, Germany, ²³Rigshospitalet, Copenhagen, Denmark, ²⁴Pediatric Rheumatology, Scleroderma Center of Pittsburgh, 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:

The new adult classifiction criteria (1) for Systemic Sclerosis (SSc) were recently published and the pediatric criteria were published in 2007 (2). None of these criteria were validated in a pediatric cohort of patients.

Methods:

We validated the adult SSc criteria in the juvenile SSc inception cohort stratified by the disease duration of the patients. We performed a latent class analysis on the adult SSc criteria for investigating the pattern of presentation of juvenile patients adjusted for disease duration.

Results:

All pediatric patients in the cohort fulfilled the adult classification criteria of proximal skin sclerosis from MCP joint. It is the main criteria of the pediatric classification system and it already gives sufficient points to be classified as SSc. Raynauds’ phenomena (73%) and nail fold changes (57%) were the most common items fulfilled by the juveniles. The pediatric patients had a mean score of 15.3 at <6 months (n=16), 15.5 at <12 months (n=31), 16.5 at < 24 months (n=62) and 17.1 at <48 months (n=86). The latent class analysis on the adult SSc criteria resulted in 3 groups of juvenile SSc patients (table 1). All patients in the first severely affected group had proximal skin sclerosis, fingertip pitting scars, nail fold changes and raynauds’ phenomena. In the mild third group (n=33), the item skin sclerosis was most important, the other criteria played a minor role in that group.

Table 1. Results of the latent class analysis on the adult SSc criteria in the inception cohort of juvenile systemic sclerosis.



	Class I	Class II	Class III
	n=16	n=37	n=33
	%	%	%

Proximal skin sclerosis	100.0	100.0	100.0
Puffy Fingers	0.0	0.0	0.0
Sclerodactyly	100.0	100.0	100.0
Digital tip ulcers	81.3	0.0	0.0
Fingertip pitting scars	100.0	44.4	36.4
Telangiectasia	0.0	0.0	0.0
Nailfold changes	100.0	78.4	12.1
PH	12.5	13.5	0.0
ISL	81.3	29.7	39.4
Raynauds	100.0	94.6	36.4
SSc AB	18.8	74.3	3.2

Conclusion:

All pediatric patients fulfilled the adult classification criteria. The cumulative score increased continuously with an increasing disease duration. The latent class analysis revealed 3 groups of jSSc patients regarding the performance of adult SSc criteria. An adaption of jSSc criteria, that patients without skin involvement can be included, would be important, to increase the number of patients with early diagnosis.

References

1. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55.

2. Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA, Jr., Lehman TJ, et al. The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007;57(2):203-12.

Disclosure: I. Foeldvari, None; J. Klotsche, None; V. Stanevicha, 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, Stanevicha 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. Performance of the Adult Systemic Sclerosis Classification in Juvenile Systemic Sclerosis Patients. Results from the Juvenile Systemic Sclerosis Inception Cohorte Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/performance-of-the-adult-systemic-sclerosis-classification-in-juvenile-systemic-sclerosis-patients-results-from-the-juvenile-systemic-sclerosis-inception-cohorte-www-juvenile-scleroderma-com/. Accessed .

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/performance-of-the-adult-systemic-sclerosis-classification-in-juvenile-systemic-sclerosis-patients-results-from-the-juvenile-systemic-sclerosis-inception-cohorte-www-juvenile-scleroderma-com/