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Lung involvement in systemic sclerosis

Doi : 10.1016/j.lpm.2010.08.006 

Paul M. Hassoun 

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Scleroderma is a multisystem disease characterized by a severe inflammatory process and exuberant fibrosis. Lung involvement is a frequent complication and a leading cause of morbidity and mortality in this syndrome. Two major pulmonary syndromes are associated with scleroderma; a pulmonary vascular disorder evolving over time into relatively isolated pulmonary arterial hypertension (PAH), and interstitial lung disease (ILD). Each syndrome, when present, is a cause of morbidity and significantly reduces survival of scleroderma patients when compared to patients free of lung complication. When pulmonary hypertension and ILD are combined, survival is further reduced. Current therapy appears to have no meaningful effect on either condition and, thus, there is a need for better understanding of underlying pathogenic mechanisms. This review focuses on clinical, diagnostic, and therapeutic features of PAH and ILD as well as other frequent but less debilitating lung complications of scleroderma.


© 2010  Elsevier Masson SAS. Tous droits réservés.
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Vol 40 - N° 1P2

P. e25-e39 - janvier 2011 Retour au numéro
Article précédent Article précédent
  • Quarterly Medical Review: Pulmonary involvement in systemic diseases
  • Marc Humbert
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  • Pleural and pulmonary involvement in systemic lupus erythematosus
  • Olga Torre, Sergio Harari

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