Lung fibrosis: new classifications and therapy

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Abstract
The recent American Thoracic Society/European Respiratory Society consensus classification of idiopathic interstitial pneumonia is equally applicable to pulmonary fibrosis associated with connective tissue disease. The most frequent histopathologic entities are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP), which is more prevalent than UIP in systemic sclerosis. The prognostic significance of NSIP is unknown in connective tissue disease, although NSIP has a better prognosis than UIP in idiopathic interstitial pneumonia. The use of computed tomography to distinguish between UIP and NSIP requires further refinement. Recent therapeutic studies have reinforced disenchantment amongst clinicians with corticosteroid and immunosuppressive regimens in UIP. UIP is increasingly regarded an “epithelial–fibrotic” disease rather than a primarily inflammatory disorder, accounting for recent widespread interest in antifibrotic agents. This conclusion should not be extrapolated to NSIP, especially in connective tissue disease. Strong circumstantial evidence of a therapeutic benefit justifies the continued use of cyclophosphamide in progressive lung fibrosis in systemic sclerosis.