Pulmonary fibrosis (PF) is a respiratory condition that causes scarring of lung tissue. The thickened tissue prevents enough oxygen from getting to the bloodstream and vital organs, causing symptoms like shortness of breath or a dry, hacking cough.
PF is more common among middle-aged and older people. It is a progressive disease, although in some patients it takes years for it to become severe.
Genetics may contribute to the development of PF, but in most cases the cause is unknown – a condition known as idiopathic pulmonary fibrosis (IPF). Each patient tends to have an individualized experience with the disease, which can progress rapidly or slowly.
Here are approved treatments for pulmonary fibrosis that can improve symptoms or slow the disease’s progression:
Ofev (nintedanib) is an approved anti-fibrotic drug the American doctors use to treat PF. In clinical trials, it slowed the decline in lung function in mild-to-moderate IPF. It works by inhibiting excess production of growth factors in IPF patients. It is taken orally twice a day. The most common side effects include diarrhea, nausea, abdominal pain, vomiting, higher levels of liver enzymes, decreased appetite, headache, weight loss, and hypertension.
Esbriet (pirfenidone) is an approved anti-fibrotic as well as anti-inflammatory drug that is used to treat IPF in the United States, Canada, Europe and Asia. In clinical trials, it slowed the progression of mild-to-moderate IPF. It is taken orally three times a day. Side effects of pirfenidone are mild. They include nausea, rash, abdominal pain, upper respiratory tract infection, diarrhea, fatigue, headache, indigestion, dizziness, and vomiting.
Corticosteroids (cortisone, prednisone) suppress the immune system and decrease inflammation. They work by mimicking the action of cortisol, a hormone produced by the adrenal glands. Corticosteroids can have many side effects, depending on the way they are administered, so they need careful monitoring.
Cyclophosphamide (brand name Cytoxan) is a chemotherapeutic agent that helps suppress inflammation. Used to treat certain forms of PF, it can be taken orally or intravenously. The most common side effects of Cytoxan include loss of appetite, inability to menstruate, skin color changes, diarrhea, hair loss, nausea, skin rashes, stomach discomfort, vomiting, and weakness.
Azathioprine (brand name Imuran) suppresses the immune system. It is used to treat pulmonary fibrosis and autoimmune diseases such as rheumatoid arthritis. It can also be used, in conjunction with other medications, to prevent the body from rejecting a transplant. Although some people respond well to Imuran, its effectiveness has yet to be confirmed in a randomized clinical trial.
Mycophenolate mofetil (brand names Cellcept and Myfortic) changes immune system functions and inhibits the proliferation of white blood cells. It is commonly used with other medications to help treat several autoimmune disorders and prevent the body from rejecting organ transplants. Scientists recently discovered that it is also an anti-inflammatory and anti-proliferative agent because it acts on some growth factors. Some of its side effects include constipation, stomach pain or swelling, nausea, vomiting, difficulty falling asleep or staying asleep, headache, and bloating.
N-acetylcysteine (NAC) is a naturally occurring antioxidant. Researchers once thought it could protect the lungs from the oxidative injury associated with some forms of PF. A clinical trial in 2014 indicated that it offers little benefit to PF patients, however. Clinical trials of NAC continue, despite that finding.
Proton pump inhibitors (Prilosec, Nexium) block acid formation in the stomach. They are used to treat the gastrointestinal problems — including acid reflux and gastrointestinal reflux disease (GERD) — that effect up to 90 percent of PF patients. Side effects are rare, but include headache, diarrhea, constipation, nausea and itching.
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