Diagnosis and Treatment of Interstitial Lung Diseases

Interstitial lung diseases compromise a group of diffuse predominantly chronic inflammatory and fibrosing disorders of the lung parenchyma. This article reviews practice-relevant publications on the diagnosis and treatment of idiopathic pulmonary fibrosis - the most common form of idiopathic interstitial lung diseases. The new patient questionnaire of the German Society of Pneumology facilitates collection of a complex case history of diverse interstitial lung diseases in individual patients. The Fleischner Society White Paper emphasize the importance of high-resolution computer tomography in the diagnosis of IPF. Histological diagnosis of the fibrotic changes is necessary in two situations: in case of CT findings indeterminate or suggestive for a diagnosis other than IPF and in case of inconsistence of medical history, laboratory findings and diagnostic imaging. The new international guidelines emphasize the importance of the interdisciplinary discussion prior to invasive diagnostic procedures, especially in the case of nonspecific interstitial lung changes. Bronchoscopy with lung cryobiopsy has gained importance in recent years. The recommendation to standardize this diagnostic method is speicified in a recently published expert opinion. The German Guideline for Idiopathic Pulmonary Fibrosis, published in 2017 suggests current therapeutic standards in this disease. The treatment with one of two registered antifibrotic drugs (pirfenidone, nintedanib) is to be started as soon as possible after the diagnosis and should be a long-term therapy. In case of intolerance or a significant progress of the disease despite the treatment, the therapy switching to an alternative drug may be considered. The combination of both therapies is not recommended. In current studies, new drugs are being tested (e.g. the serum protein pentraxin 2, antibodies against the growth factor CTGF). On the other hand, the efficacy of the registered antifibrotic medicaments, nintedanib and pirfenidone, in interstitial lung diseases other than idiopathic pulmonary fibrosis is being evaluated. © Georg Thieme Verlag KG, Stuttgart. New York.