Rheumatology 2000; 39: 556-559
© 2000 British Society for Rheumatology
Paediatric Rheumatology |
Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey
University Children's Hospital, Hamburg, Germany,
1 Medical University of Tbilisi, Georgia, Russia,
2 Children's Hospital of Eastern Ontario, Ottawa, Canada,
3 Hospital de Pediatria Pedro de Elizalde, Buenos Aires, Argentina,
4 Federal University of Rio de Janeiro, Rio de Janeiro, Brasil,
5 McMaster University, Hamilton, Ontario, Canada,
6 Department of Rheumatology, Lund University Hospital, Sweden,
7 Rheumatology Unit, University of Florence, Italy,
8 St Josef Stift, Sendenhorst,
9 University Children's Hospital, Würzburg,
10 Rheumakinderklinik, Garmisch-Partenkirchen, Germany,
11 Centre for Paediatric Rheumatology, University Gent, Belgium,
12 Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands,
13 Children's Hospital, University of Helsinki, Finland,
14 Hôpital Necker Enfants Malades, Paris, France,
15 Paediatric Clinic, Institute of Rheumatology, Warszawa, Poland,
16 Hospital de Pediatria, Buenos Aires, Argentina,
17 Rheumatism Foundation Hospital, Heinola, Finland,
18 Paediatric Rheumatology Clinic, Department of Pediatrics, University of Ioannina, Greece and
19 Centro di Rheumatologia Pediatrica, University of Padova, Padova, Italy
Abstract
Objective. To increase the current knowledge of the outcome of juvenile systemic sclerosis (jSSc), which is currently limited.
Methods. In order to investigate the patient outcome and prognostic factors, starting October 1994, we distributed questionnaires to 324 paediatric rheumatology centres.
Results. Until 15 May 1998 responses from 46 centres were received, 34 of which returned completed questionnaires on a total of 135 patients. One hundred and twenty-two of the 135 patients were Caucasian, 100 were female. The mean age at disease onset was 8.8 yr (S.D. ± 3.3 yr). The mean disease duration at the last follow-up was 5 yr(S.D. ± 3.3 yr). At the last follow-up the disease was still active and required medication in 82 patients, 36 had inactive disease on medication, and 16 were in remission. Ninety per cent of the living patients were fully active in daily life at the last follow-up. Eight of the 135 patients had died. These patients had a median age at onset of the disease of 10.5 yr (range 6.7–15.8 yr). The median disease duration until death was 2 yr (range 1–8 yr). The causes of death were heart failure (five), renal failure (one), sepsis (one) and in one case the cause was not defined. The 1 yr survival rate was 99%, the 2 yr was 97% and the 4 yr was 95%.
Conclusions. At a mean follow-up of 5 yr, the current results show a favourable outcome in most patients with childhood onset jSSc and a significantly better survival than in the adult SSc patients.
KEY WORDS: Childhood, Outcome, Mortality, Morbidity, Survival.
Notes
Correspondence to: I. Foeldvari, University Children's Hospital, Martinistrasse 52, D-20246 Hamburg, Germany.
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