Idiopathic pulmonary fibrosis and other progressive pulmonary fibroses. Update of the guidelines on diagnostics and treatment

A wide selection of medicinal treatment options are currently available for the treatment of patients with interstitial lung diseases (ILD), so that an early and correct diagnosis is of significant importance. Therefore, in the new German guidelines consensus statements on the diagnostics and courses of the various ILDs were formulated, so that the available treatment strategies are used in an optimized way in the appropriate patient populations. In the first part of the guidelines the current state of knowledge and the evidence on idiopathic lung fibrosis (IPF), which is considered to be a model disease for fibrosing ILD, were updated and supplemented by the experiences of experts. The effectiveness of antifibrotic treatment could be confirmed in many new post hoc analyses and long-term data and further strengthened by real-world observations. The treatment with nintedanib and pirfenidone is currently the treatment of choice for patients with IPF. Less clear is the situation with other forms of ILD that have a pronounced inflammatory component and can develop a progressive fibrosing disease course with varying frequencies. Therefore, the second part of the guidelines deals with progressive fibrosing interstitial lung diseases (PF-ILD) or synonymously progressive pulmonary fibrosis (PPF), which give rise to the indications for an antifibrotic treatment with nintedanib, when they fulfil the newly defined criteria for progression. In contrast, ILDs that do not take a progressive fibrosing course are usually treated with various anti-inflammatory drugs.