Diffuse Parenchymal Lung Disease (DPLD), or Interstitial Lung Disease (ILD), comprises a group of over 200 different disorders, which are mainly characterized by progressive scarring of the lung architecture and often result in respiratory failure. DPLD occurs in children and adults secondary to acute or chronic lung injury provoked by the inhalation of toxic gases or dusts, as part of systemic diseases (e.g. connective tissue disorders), or as a result of therapeutic measures (e.g. mechanical ventilation or chemotherapy). In a significant proportion of patients, however, the cause remains unknown; this is called Idiopathic Interstitial Pneumonia (IIP), which also comprises Idiopathic Pulmonary Fibrosis (IPF).
Although different in origin, the course of many DPLDs is similar as the disease progresses and is associated with poor prognosis. Only in recent years have antifibrotic treatments for IPF become available. These do not entirely prevent the disease from progressing but may slow down scarring in the lungs, which can be prognostically relevant. The only curative treatment, however, is lung transplantation. To develop new diagnostic strategies and causal treatment options, it is indispensable to gain deeper insights into the early and essential underlying disease mechanisms. More specific and earlier identification of disease-relevant alterations, which makes it possible to assess the risks, should enable the development of individualized therapeutic concepts also in the field of DLPDs. The DPLD Disease Area of the DZL brings together and increasingly links internationally renowned experts for both pediatric and adult forms of DPLD. This makes it possible to better identify reparative as well as regenerative processes and develop both diagnostic and therapeutic options, from the newborn to the elderly patient.