Idiopathic Pulmonary Fibrosis: A New Histopathologic Classification and Clinical Aspects

peer reviewedIdiopathic pulmonary fibrosis (IPF) comprises different interstitial lung diseases of unknown origin. They are classified into four groups based on pathologic criteria: UIP, DIP, AIP and NSIP. This classification helps in terms of prognosis and treatment. In addition to history, physical examination and common investigations (chest X ray, pulmonary function testing), a bronchoalveolar lavage (BAL), a high-resolution CT scan and ideally a open-lung biopsy are needed to establish a precise diagnosis. The patients are usually treated by immunosuppressive agents, and mainly by corticoids. However, in UIP, lung transplantation is the only way to improve survival