Rheumatology Advance Access published online on October 14, 2008
Rheumatology, doi:10.1093/rheumatology/ken388
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Factors affecting survival in juvenile systemic sclerosis
1Department of Paediatrics, University of Padua, Padova, Italy 2Department of Pediatrics, Cerrahpasa Tip Fakultesi, Istanbul, Turkey 3Department of Pediatrics, Fondazione IRCCS Policlinico S. Matteo, Pavia 4Clinica Pediatrica II De Marchi Milan, Milan, Italy 5Instituto Nacional de Rehabilitacion-Servicio de Reumatologia, Tlalpan, Mexico DF, Mexico 6First Faculty of Medicine and General Faculty Hospital, Praha, Czech Republic 7Department of Pediatrics, Universidade Federal de Sao Paulo, Sao Paulo, Brazil 8Unidad de Reumatologia Hospital Sor Maria Ludovica, La Plata, Argentina and 9University Hospitals, Copenhagen, Denmark.
Correspondence to:
G. Martini, Department of Pediatrics, University of Padua, Via Giustiniani 3, 35128 Padua, Italy. E-mail: martini{at}pediatria.unipd.it
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Abstract |
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Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).
Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.
Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).
Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality.
KEY WORDS: Scleroderma, Mortality, Systemic sclerosis, Child, Outcome
Submitted 23 November 2007;
revised version accepted 5 September 2008.
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