Pulmonary fibrosis refers to all lung diseases characterized by an increase and redistribution of lung connective tissue (scarring). As these lung diseases are rare, they usually require complex testing and treatment by specialists.
Fibrosis results in a loss of lung elasticity and volume. This primarily leads to increased shortness of breath and often to coughing. The physical capacity of affected patients progressively worsens.
Pulmonary fibrosis can have many different causes. Pulmonary fibrosis induced by known causes (e.g. triggered by medication or connected to rheumatic diseases) is differentiated from lung fibrosis caused by unknown reasons (idiopathic pulmonary fibrosis).
In addition to radiological procedures (usually high-resolution computed tomography of the lungs) and pulmonary function tests, the cornerstones of diagnosis include endoscopic or surgical lung tissue biopsies.
There are no specific causal therapies available for the treatment of idiopathic pulmonary fibrosis. Antifibrotic treatment is possible for patients with mild to moderate idiopathic pulmonary fibrosis. This slows the loss of lung function and thus the progression of the disease. An application for the reimbursement of costs of the medication must be completed for each individual case.
International guidelines recommend an interdisciplinary diagnosis of idiopathic pulmonary fibrosis. Each individual case is therefore discussed jointly with specialized physicians and specialists in radiology, pathology and pulmonology.