Due to the wide range of interstitial lung diseases (ILD) with varying prognoses, accurate diagnosis and identification of the underlying cause are essential in patients with ILD to enable targeted therapy. In approximately 50% of cases, an underlying cause can be identified [1]. Once the trigger has been successfully determined, avoidance or elimination of the causative agent represents the key therapeutic intervention. If no identifiable or removable cause can be found, or if exposure cessation alone does not lead to recovery, further treatment follows principles similar to those applied in ILDs of unknown etiology. In this context, distinguishing between inflammatory and fibrotic processes is often challenging [2]. Depending on the disease course, anti-inflammatory and/or antifibrotic therapy may be indicated [3].
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