Abstract
Idiopathic pulmonary fibrosis is uncurable and fatal disease manifested by fibrotic remodeling of the lung interstitium. Advances in understanding the pathogenesis of the disease have led to doubts about the corticosteroid and immunosuppressive therapy.
The outputs from the PANTHER study even showed an increased mortality from combined immunosuppression compared to placebo. Therefore, completely new drugs with antifibrotic effects have been developed.
There are currently two available - pirfenidone and nintedanib. Both have a comparable effect in reducing the decline in lung function and prolonging survival.
In addition, N-acetylcysteine can be used in appropriately indicated patients. A serious therapeutic issue is the management of exacerbations of idiopathic pulmonary fibrosis.
Although controversial, methylprednisolone pulses are usually applied.