Ružena Tkáčová, Štefan Tóth
Summary: Current classification of idiopathic interstitial pneumonias (IIP) includes seven clinico-radiologic-pathologic entities: idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. The need for dynamic interaction between pathologists, radiologists, and pulmonologists to diagnose accurately these disorders is emphasized. Despite major accomplishments in our understanding the pathogenesis of lung fibrosis, the diagnosis and management of patients with IIP continues to pose significant challenges. Corticosteroids represent the core of the treatment of IIP and their effectiveness is assessed in 3-6 months intervals. Based on the chest x-ray, high-resolution computerized tomography, pulmonary function tests, bronchoalveolar lavage, and laboratory and clinical parameters the clinician has to decide whether to continue or discontinue the corticosteroid therapy, or whether to change the treatment regimen to corticosteroids plus azathioprine or cyclophosphamide.