Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis (IPF) is the most common of the spontaneously occurring diffuse parenchymal lung diseases also known as idiopathic interstitial pneumonias (IIPs).
IPF is a type of rare lung disease in which the tissue around the alveoli (air sacs) within the lung become thickened and scarred (often referred to as fibrosis). The scarring of the lungs causes the lungs to become stiff and impairs the ability of the oxygen to move into the blood stream where it is needed to be transported to the entire body.
Idiopathic means no known cause and as such, the reason for someone to get IPF is unknown. In Australia, it is estimated that approximately 1,250 people per year are diagnosed with this condition.
Early in the disease, symptoms can be very mild. Common symptoms include dyspnea (shortness of breath), dry persistent cough (occasionally people may produce clear phlegm) and reduced exercise capacity. Other, less specific symptoms include feeling tired and gradual unintended weight loss.
Current treatment options include:
- Medications – there are currently 2 anti-fibrotic medicines (Pirfenidone and Nintedanib) which have been shown to help slow the rate of progression.
- Oxygen therapy
- Lung Transplantation – may be an option in severe cases but is determined on a case-by-case basis.
