Idiopathic pulmonary fibrosis (IPF): what you need to know

Quick fact: IPF is a scarring lung disease that slowly makes it harder to breathe. People often notice shortness of breath with exercise, a dry cough, and tiredness that doesn’t go away. IPF isn’t caused by a single known trigger—hence the word “idiopathic”—but early recognition and care can slow the decline and improve quality of life.

How doctors diagnose IPF

If you or a loved one has persistent breathlessness or a chronic dry cough, a doctor will usually start with a history and a lung exam. Important tests include pulmonary function tests (measuring FVC and DLCO), a high-resolution CT scan of the chest, and sometimes blood work to rule out other causes. In unclear cases, a bronchoscopy or surgical lung biopsy may be suggested, but many patients are diagnosed based on CT plus lung function.

Listen for “velcro” crackles on exam and watch for clubbing of the fingers—these are clues doctors use. Track symptoms and any exposures (smoke, birds, workplace dust) before appointments; that makes the work-up faster and more accurate.

Treatments and daily care

There’s no cure for most cases of IPF, but treatments can slow progression and ease symptoms. Two proven antifibrotic drugs—pirfenidone and nintedanib—are commonly used. They don’t reverse scarring, but they can reduce the rate of lung function decline. Both have side effects (nausea, diarrhea, liver changes), so regular follow-up and blood tests matter.

Other helpful steps: supplemental oxygen if your oxygen levels drop, pulmonary rehabilitation to build strength and reduce breathlessness, flu and pneumonia vaccines, and careful management of other conditions like heart disease. If breathlessness is severe, palliative care teams can help with symptom control and planning.

For some people, a lung transplant is an option. Transplant evaluation should start early—while you’re still active enough to benefit. Ask your specialist about clinical trials too; new treatments are always being tested.

Practical day-to-day tips: conserve energy (break tasks into small steps), use breathing techniques (pursed-lip breathing helps), avoid smoke and pollutants, stay active within your limits, and eat well to keep strength up. Keep an updated medication list and bring it to every visit.

Know when to call your doctor: sudden worsening breathlessness, fevers, chest pain, or new swelling in the legs. These can be signs of infection, blood clots, or heart issues that need prompt attention.

Living with IPF is challenging, but good care and planning make a big difference. Find a respiratory specialist, ask about antifibrotic therapy if it fits your case, consider pulmonary rehab, and connect with local support groups to share tips and stay informed.