Treatment of pulmonary fibrosis

Treatment of pulmonary fibrosis

Good protection against the causing occupational agents and avoidance of smoking and causing medications is an effective prevention.

When de disease has been diagnosed, a few non-drug options are available. Oxygen supply improves the quality of life and exercise capacity. For some (younger) patients lung transplantation is the best treatment option.Medical treatment options are very limited. Some types of lung fibrosis can respond to corticosteroids (prednisone) sometimes in combination with immunosuppressants (cyclosporin, azathioprine, cyclophosphamide and methotrexate). The goal of these drugs is to decrease the inflammation and subsequent scarring.

Prednisone and other immunosuppressant medications cause serious side effects, including diabetes, glaucoma, reduced production of red blood cells, skin cancer and lymphoma.


For that reason, treatment is usually discontinued if there's no improvement after six months.

The antifibrotic drug pirfenidone has been shown to improve lung function and prevent destruction of lung tissue. In vitro, it inhibits TGFβ-stimulated collagen synthesis, decreases the extracellular matrix, and blocks fibroblast proliferation.

One study (Ifigenia) showed some effect of acetylcysteine (as a radical scavenger) in combination with prednisone and cyclophosphamide.

The role of endothelin receptor antagonists (bosentan) is currently under evaluation. In an animal model, treatment with bosentan decreased the amount of collagen deposition.