Factors affecting survival in juvenile systemic sclerosis

G. Martini, F. Vittadello, Ö Kasapçopur, S. Magni Manzoni, F. Corona, C. Duarte-Salazar, D. Nemcova, C. A. Len, S. M. Garay, S. Ullman, F. Zulian, Ruben Cuttica, Ricardo Russo, Graciela Espada, María Teresa Apaz, Maria Odete Hilario, Ana Paola Lotito, Claudia Machado, Flavio Sztajnbok, Sheila Knupp F De OliveiraBernadete de I Liphaus, Clovis A. Almeida da Silva, Traudel Saurenmann, Susan Nielsen, Rebecca Ten Cate, Lisette Van Suijlekom-Smit, Hermann Girschick, Ivan Foeldvari, Gerd Horneff, Carine Wouters, Paul Galea, Patricia Woo, Rosa Merino, Rosa Roldan Molina, Maria Luz Gàmir, Joan Ros, Michel Fischbach, Angelo Ravelli, Antonella Buoncompagni, Elisabetta Cortis, Fernanda Falcini, Maria Giannina Alpigiani, Clodoveo Ferri, Andrey Scegolev, Dana Němcová, Ilonka Orbàn, Henryka Mazur Zielińska, Elzbieta Musiej-Nowakowska, Tadej Avcin, Pasic Srdjan, Roksanda Stojanovic, Gordana Susic, Mukamel Masha, Yoseph Uziel, Galina A. Lyskina, Sulaiman Al-Mayouf, Anne Eberhard, Gloria Higgins, Lynn Punaro, Audrey Nelson, Robert Sundel, Terry L. Moore, Balu H. Athreya, Thomas Lehman

Research output: Contribution to journalArticle

50 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)119-122
Number of pages4
JournalRheumatology
Volume48
Issue number2
DOIs
StatePublished - 2009

Keywords

  • Child
  • Mortality
  • Outcome
  • Scleroderma
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

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    Martini, G., Vittadello, F., Kasapçopur, Ö., Magni Manzoni, S., Corona, F., Duarte-Salazar, C., Nemcova, D., Len, C. A., Garay, S. M., Ullman, S., Zulian, F., Cuttica, R., Russo, R., Espada, G., Apaz, M. T., Hilario, M. O., Lotito, A. P., Machado, C., Sztajnbok, F., ... Lehman, T. (2009). Factors affecting survival in juvenile systemic sclerosis. Rheumatology, 48(2), 119-122. https://doi.org/10.1093/rheumatology/ken388