"Even a disease as deadly as idiopathic pulmonary fibrosis (IPF) can start out as just a cough. But as the condition progresses, a person might experience up to 500 respiratory spasms per day. Breathing becomes difficult, as does sleeping. A chronic lack of oxygen increases blood pressure in the lungs. Within 3-5 years of diagnosis, if untreated, a person will experience respiratory failure and die."
"Attempts to treat the condition - or at least alleviate its symptoms - have largely focused on fibrosis, the formation of scar tissue around the tiny air sacs in the lung known as alveoli. So far, it's been a rough ride. Treatment with a combination of anti-inflammatory drugs, which was the standard of care for many years, is now not advised because a clinical study in 2012 concluded that it not only failed to improve outcomes, but also increased mortality."
Idiopathic pulmonary fibrosis often begins with a mild cough and progresses to severe respiratory impairment, including as many as 500 respiratory spasms per day and difficulty sleeping. Chronic low oxygen increases pulmonary blood pressure and can cause respiratory failure and death within three to five years if untreated. The cause is unclear but likely involves genetic and environmental factors. Treatment efforts target fibrosis around alveoli. Combination anti-inflammatory therapy was found harmful in 2012. Two FDA-approved drugs, nintedanib and pirfenidone, are now standard; nintedanib slows forced vital capacity decline but causes side effects and has not shown mortality reduction.
Read at Nature
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