Lung fibrosis: new classifications and therapy

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 f requent histopathologic entities are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP), which is more prevalent than UI P in systemic sclerosis. The prognostic significance of NSIP is unknown in connective tissue disease, although NSIP has a better prognosis than UP in idiopathic interstitial pneumonia. The use of computed tomography to distin guish between UIP and NSIP requires further refinement. Recent therapeutic studies have reinforced disenchantment amongst clinicians with corticostero id and immunosuppressive regimens in UIP. UP is increasingly regarded an "e pithelial-fibrotic" disease rather than a primarily inflammatory disorder, accounting for recent widespread interest in antifibrotic agents. This conc lusion 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 syste mic sclerosis. Curr Opin Rheumatol 2001, 13:500-504 (C) 2001 Lippincott Wil liams & Wilkins, Inc.