Neumopatía intersticial en la esclerosis sistémica

Interstitial lung disease (ILD) occurs frequently in patients with systemic sclerosis (SSc), with a burden of significant morbidity and mortality. SSc-ILD resembles idiopathic pulmonary fibrosis (IPF) in many respects, including a restrictive ventilatory defect that results from an active fibrosing process in the lung interstitium, a ground glass opacification on high resolution computed tomography, and inflammatory cells in bronchoalveolar lavage fluid. Differences in the classification of lung histo-pathology are noted frequently and may account for some of the differences in outcome for patients with SSc-ILD and IPF. Optimal treatment of SSc-ILD remains to be determined, but cyclophosphamide has been reported to be effective in several open observational studies. Results from the Scleroderma Lung Study are expected to add new insights into the optimal therapy for SSc-ILD.