Histopathological pattern of progressive pulmonary fibrosis could be seen in many different fibrotic lung interstitial diseases. Exact diagnosis is crucial for precise therapy, moreover, different diseases have different prognosis.
The most important disorders in this group are idiopatic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis, and their separation is crucial because of totally different treatment of the patients. The aim of this review is to sum up the most important characteristics of usual interstitial pneumonia, histopathological pattern of idiopatic pulmonary fibrosis, and fibrotic hypersensitivity pneumonitis and provide a practical work-up for precise diagnostics of these diseases in the frame of effectively cooperating multidisciplinary team.