Idiopathic Pulmonary Fibrosis (IPF)

• Idiopathic pulmonary fibrosis (IPF) is a specific form of progressive fibrosing interstitial lung disease that can cause pneumonia.
• Idiopathic pulmonary fibrosis has no known cause.
• Symptoms of idiopathic pulmonary fibrosis include a six-month history of
  • shortness of breath and/or cough,
  • fatigue,
  • mild fevers,
  • weight loss,
  • joint discomfort,
  • muscle pain,
  • clubbing fingers,
  • ankle swelling, and
  • in some patients, a heart murmur.
• Stages of idiopathic pulmonary fibrosis are not standardized, but some staging systems are designed to include certain criteria: traditionally, idiopathic pulmonary fibrosis has been staged as mild, moderate, or severe.
• Doctors who treat idiopathic pulmonary fibrosis are primary care physicians, pulmonologists, gastroenterologists, and transplant surgeons.
• The diagnosis of idiopathic pulmonary fibrosis is one of exclusion of other interstitial lung diseases. In addition, high-resolution computed tomography (CT scans) and surgical biopsy of the lung tissue help to confirm the diagnosis.
• Treatment of idiopathic pulmonary fibrosis may involve medications, supportive therapy, and/or lung transplantation.
• Guidelines from many organizations recommend the use of pirfenidone (Esbriet) and nintedanib (Ofev).
• Lung transplantation is the treatment option that does show survival benefit over medical therapy.
• Because idiopathic pulmonary fibrosis is idiopathic (no known cause), researchers are not sure how to prevent the disease, but suggest that avoiding smoking and reducing or eliminating exposure to toxins or fumes that may damage the lungs may decrease risk of idiopathic pulmonary fibrosis.
• Life expectancy is about 2 to 5 years. Patients who undergo lung transplant have a mortality rate of about 50% to 56% after five years so the prognosis of idiopathic pulmonary fibrosis is usually fair to poor. Patients and their families are urged to contact support groups included in this information.

Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of progressive fibrosis causes interstitial pneumonia, usually in older adults and is limited to the lungs. Fibrotic (scar-like) tissue develops in the airways of the lungs, making it difficult for the body to get the oxygen it needs. Idiopathic pulmonary fibrosis differs from pulmonary fibrosis in that the cause is unknown whereas there are known causes of pulmonary fibrosis.

This article will discuss idiopathic pulmonary fibrosis. Pulmonary fibrosis is the subject of another article. Though not very common, idiopathic pulmonary fibrosis is considered to be a debilitating, incurable, and terminal disease by many health-care professionals and reasearchers.