Interstitial lung disease (ILD) is a growing group of lung disorders with diverse causes and clinical manifestations.[1][5] Research focuses on understanding the mechanisms of lung fibrosis, especially the role of signaling pathways and alveolar epithelial cell damage.[1] In diagnostics, new biomarkers from blood, respiratory tract and imaging diagnostics are applied, which improve the detection and monitoring of the disease.[1] Among the most common forms of ILD is idiopathic pulmonary fibrosis (IPF), which has no known cause and is diagnosed in approximately 50,000 patients annually.[6] In the treatment of IPF, antifibrotic therapy was introduced in 2011, which slows the progression of the disease and extends the life of patients by an average of 2.5 years.[1][3] The simultaneously registered drugs pirfenidone and nintedanib have shown a significant slowing of lung function deterioration and a reduction in mortality.[1][2] New approaches include precision medicine, individualized treatment and cell therapies that promise to improve the quality of life of patients with this serious disease.[1]