Is there a different presentation of juvenile systemic diffuse and limited subset? data from the juvenile scleroderma inception cohort.

Ivan Foeldvari, Jens Klotsche, Ozgur Kasapcopur, Amra Adrovic, Kathryn S. Torok, Maria Teresa Terreri, A. P. Sakamoto, Valda Stanevicha, F.R. Sztajnbok, Jordi Anton, Brian Feldman, Ekaterina Alexeeva, M. Katsikas, Vanessa Smith, Tadej Avcin, Edoardo Marrani, Mikhail Kostik, Thomas Lehman, Walter‐Alberto Sifuentes‐Giraldo, Natalia Vasquez‐CanizaresSimone Appenzeller, Mahesh Janarthanan, M. Moll, D. Nemcova, Anjali Patwardhan, Maria Jose Santos, Sujata Sawhney, Dieneke Schonenberg‐Meinema, Cristina Battagliotti, Lillemor Berntson, B. Bica, Jurgen Brunner, P. Costa Reis, Despina Eleftheriou, L. Harel, Gerd Horneff, Tilmann Kallinich, D. Kaiser, D. Lazarevic, Kirsten Minden, S. Nielsen, Farzana Nuruzzaman, Yosef Uziel, Nicola Helmus

Research output: Contribution to journalMeeting Abstractpeer-review


Introduction: Juvenile systemic scleroderma (jSSc) has a prevalence
of 3 per 1 000 000 children. There are limited data regarding the
clinical presentation of jSSc. The Juvenile Systemic Scleroderma
Inception Cohort (jSScC) is the largest multinational registry that
prospectively collects information about jSSc patients.
Objectives: To assess of there is a difference in the clinical
characteristics in diffuse and limited subtype jSSc patients at the
time of inclusion in the jSScC
Methods: Patients were included, if they fulfilled the adult ACR/
EULAR classification criteria for systemic scleroderma, if they
presented the first non-Raynaud symptom before 16 years of age
and were younger than 18 years of age at time of inclusion. Patients’
characteristics at time of inclusion were evaluated.
Results: Until December 2019 hundred fifty patients were included,
83% of them being Caucasian and 80% female. The majority had the
diffuse subtype (72%) and 17% of all jSSc had overlap features. The
mean age of first presentation of Raynaud´s phenomenon was 9.8
years in the diffuse subtype (djSSc) and 10.7 years in the limited
subtype (ljSSc) (p=.197). The mean age at first non-Raynaud’s symptoms was 10.0 years in the djSSc and 11.2 years in the ljSSc (p=
0.247). Mean disease duration at time of inclusion was 3.4 years in
the djSSc and 2.4 years in the ljSSc group.
Significant differences were found between the groups
regarding mean modified Rodnan skin score, 18.2 in the djSSc vs 6.2
in the ljSSc (p=0.02); presence of Gottron´s papulae (djSSc 30% vs
ljSSc 13%, p=0.43);presence of teleangiectasia ( djSSc 42% vs 18%
ljSS, p=0.01); history of ulceration (djSSc 42% vs 18% ljSSc,p=0.008); 6
Minute walk test below the 10th percentile ( djSSc 85% vs ljSSc 54%,
p=0.044), total pulmonary involvement ( djSSc 49% vs ljSSc 31%, p=0.045), cardiac involvement (ljSSc 17% vs djSSc 3%, p=0.002). djSSc
patients had significantly worse scores for Physician Global
Assessment of disease activity compared to ljSSc patients (VAS 0-
100) (40 vs 15) (p=0.001) and for Physician Global Assessment of disease damage (VAS 0-100) (36 vs 17) (p=0.001).
There were no statistically significant differences in the other
presentations. Pulmonary hypertension occurred in approximately
6% in both groups. No systemic hypertension or renal crisis was
reported. ANA positivity was 90% in both groups. Anti-Scl70 was
positive in 35% in djSSc and 36% in the ljSSc group. Anticentromere
positivity occurred in 3% in the djSSc and 7% in the ljSSc group.
Conclusion: In this unique large cohort of jSSc patients there were
significant differences between djSSc and ljSSc patients at time of
inclusion into the cohort regarding skin, vascular, pulmonary and
cardiac involvement. According to the physician global scores the
djSSc patients had a significantly more severe disease.
Supported by the "Joachim Herz Stiftung"
Original languageEnglish
Pages (from-to)44
JournalPediatric Rheumatology
Issue numberSuppl.2
Publication statusPublished - 2020
Externally publishedYes
Event26th European
Paediatric Rheumatology Congress
Duration: 23 Oct 202026 Oct 2020
Conference number: 26

Field of Science

  • 3.2 Clinical medicine

Publication Type

  • 3.4. Other publications in conference proceedings (including local)


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