Pulmonary fibrosis is a serious disease characterized by thickening and scarring (fibrosis) of the lungs. The development of fibrosis in the lungs makes it difficult for oxygen to pass through the walls of the air sac and into the bloodstream. Different pulmonary fibrosis types all lead to scarring in the lungs but can be caused by different factors.

Idiopathic pulmonary fibrosis

Several identifiable diseases and conditions can lead to scarring of the lungs, but the majority of PF cases are caused by unknown reasons. These cases are diagnosed as idiopathic pulmonary fibrosis. The translation for “idiopathic” is “unknown.” Because so many cases of pulmonary fibrosis are caused by factors that cannot be confirmed, this type of the disease is more difficult to treat. Currently, two U.S. Food and Drug Administration (FDA) approved therapies (Esbriet and OFEV) are prescribed to slow the progression of the disease. Still, IPF remains a very serious condition with patient mortality three to five years after diagnosis. The primary complication of IPF is respiratory failure, but pulmonary hypertension, heart failure, pneumonia, and lung cancer can also be complications. IPF is considered a rare disease. Only about 13 to 20 individuals per every 100,000 people worldwide are diagnosed with the disease.

Familial pulmonary fibrosis

In some cases, doctors observe a pattern of pulmonary fibrosis in families, which suggests that genetics may cause PF in certain diagnosed patients. Those types of cases are referred to as familial pulmonary fibrosis. The familial form of PF is more rare than IPF. Only about 100 families have been confirmed as genetically passing on the disease (based on the presence of PF in two or more family members).

Familial pulmonary fibrosis is as serious as IPF, but a patient who has a genetic and historical pattern in their family gives doctors an opportunity to provide an earlier diagnosis that could led to a better prognosis.

Different types of pulmonary fibrosis according to stage

Pulmonary fibrosis types can be divided into stages. Based on pulmonary function tests, the traditional stages of PF are mild, moderate, severe, and very severe. Physicians use staging to monitor the disease, advise patients and loved ones about PF progression, define prognosis, and determine a therapeutic approach.

Other pulmonary fibrosis types

Other types of pulmonary fibrosis include idiopathic nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) and sarcoidosis.

Causes for PF can be autoimmune diseases such as rheumatoid arthritis and systemic scleroderma; certain muscle diseases; occupational and environmental exposure to toxic substances including asbestos, silica, coal dust, beryllium, hard metal dusts, animal proteins, bacteria or molds; and various therapies, medications, and drugs.

Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

  1. https://www.nhlbi.nih.gov/health/health-topics/topics/idiopathic-pulmonary-fibrosis
  2. https://www.ncbi.nlm.nih.gov/pubmed/23835999
  3. http://pulmonaryfibrosismd.com/stages-of-idiopathic-pulmonary-fibrosis/
  4. http://www.medicinenet.com/pulmonary_fibrosis/article.htm