It can eventually become severe. The chest x-ray shows small linear opacities at the lung bases and the lungs have the appearance of a honeycomb. This honeycombing pattern represents cystic scarring and destruction of the lung. No medication has yet been developed that will stop or reverse the fibrosing process. Long-term oxygen therapy has been proven as an effective treatment for preventing the development of heart failure as the severity of the disorder worsens.
Pulmonary rehabilitation can be an effective part of management.
Lung transplantation has been successful in some patients. In the near future, support from the Epler Research Fund will be utilized for detecting the sequence of cellular events causing pulmonary fibrosis and for testing new treatments. Current support is used for developing the interactive personal management program for patients with idiopathic pulmonary fibrosis.