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Predictors of end stage lung disease in systemic sclerosis
  1. V Steen
  1. Georgetown University, Washington DC, USA
  1. Correspondence to:
    Professor V Steen;

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Recognition of the two types of lung disease is important because their predictors and treatment differ

Pulmonary disease is now the leading cause of death in patients with scleroderma. Of the 2000 patients in the Pittsburgh scleroderma databank, 211 patients have died of lung disease over the past 20 years. One hundred and thirteen died from isolated pulmonary hypertension and 98 died of pulmonary interstitial fibrosis. These account for 21.5% of the 981 deaths and 44% of the scleroderma related deaths. Recently, progress has been made in the treatment of specific lung problems in scleroderma so it is imperative that we have a better understanding of the predictors of these complications. Most important is the acknowledgement and recognition that there are two types of lung disease in scleroderma which are very different in their pathogenesis, clinical associations, predictive factors, and treatment. The investigators of “Predictors of end stage lung disease in a cohort of patients with scleroderma” in this issue of the Annals admit that a limitation of their study was that they were unable to separate their patients into the two types of lung disease.1 By combining these two types of lung disease they were unable to identify factors that would have likely been predictors of one of the forms of lung disease. It is extremely important to look carefully at the two types of lung disease individually and not lump them all together as end stage lung disease.


Pulmonary interstitial fibrosis is felt to result from an alveolitis, which leads to interstitial fibrosis.2 In some patients there may be a significant vascular component, as there is in other manifestations of the disease. However, the interactions of these two components is incompletely understood and thus, we will focus primarily on the inflammation and fibrosis component. In interstitial …

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