If you have a bunch of ordinary-seeming symptoms that come and go, like acid reflux, shortness of breath, or a cough that just won’t quit, they may just be because you had a stressful dinner of spicy Indian food with your in-laws while you’re still getting over the flu. But doctors warn that IPF symptoms, like these and more, may go overlooked.
Meet the expert: Nicole Ng, M.D., associate director of the Interstitial Lung Disease Program at Mount Sinai Health System
If these symptoms keep popping up and are accompanied by others, it’s a good idea to make an appointment with your doctor so they can figure out if there’s more going on. While these daily annoyances are often nothing to worry about in themselves, together they may mean you could have a lung condition such as idiopathic pulmonary fibrosis (IPF).
What is IPF?
IPF is one of more than 200 lung conditions that fall under the umbrella of interstitial lung disease (ILD). They all tend to affect the space around the air sacs (alveoli) in the lungs and cause inflammation and/or scarring, which makes the lungs stiffer and less able to move oxygen into the bloodstream.
Some types of ILD are caused by environmental exposure to certain toxins; others occur in people with autoimmune conditions that may involve the lungs, such as rheumatoid arthritis; there are other causes as well.
But when a lung scan shows a characteristic pattern of scarring with no clear cause, the diagnosis may be IPF, explains Nicole Ng, M.D., associate director of the Interstitial Lung Disease Program at Mount Sinai Health System. (The “I” is for “idiopathic,” which means “with no known cause.”) Around 50,000 Americans are diagnosed with IPF each year.
Who gets IPF?
IPF commonly affects people who are older, and because symptoms develop gradually, they may simply get chalked up to the effects of aging. “You might notice that things you used to do easily, like taking an evening stroll and doing chores around the home, slowly become harder,” and so you need to take a break, says Dr. Ng.
Also, early symptoms of IPF may be common and nonspecific, so patients can be misdiagnosed or go undiagnosed for months or even years (they’re often given antibiotics for pneumonia numerous times before getting the correct diagnosis, says Dr. Ng). That’s why it’s important to follow up if you’re not feeling better, so other possible causes can be considered.
IPF is most often diagnosed in people in their 60s and 70s, and it occurs more in men than in women, says Dr. Ng, adding, “Smoking is a known risk factor, so smoking cessation is strongly recommended.” Risk is also higher if a family member has IPF.
IPF symptoms
IPF symptoms may include:
- Shortness of breath during exercise or daily activities
- A dry cough that doesn’t
go away - Severe or persistent fatigue
- Unexplained weight loss
- Clubbing, a widening
and rounding of the tips
of the fingers or toes
Treating IPF
While there’s no cure for IPF, starting treatment early may help slow its progression, says Dr. Ng. A treatment plan may include:
Antifibrotics
These medications slow the loss of lung function and are the only ones approved specifically for IPF. “Until last year, there were only two FDA-approved antifibrotics,” nintedanib and pirfenidone, Dr. Ng explains. But a third, nerandomilast (Jascayd), was approved last October. “It’s very encouraging for us to finally have another option,” she says.
Pulmonary rehab
This therapy focuses on exercise training, breathing, and energy-conserving techniques and may also include nutrition counseling. The goal is to teach those with IPF how to improve their lung capacity so they may have a better quality of life.
Oxygen therapy
As the disease progresses, some people with IPF need supplemental oxygen during activities that make them short of breath, such as walking and climbing stairs; in later stages of the condition, they can come to rely on it more.
Treatments for associated conditions
Reflux, sleep apnea, and pulmonary hypertension commonly appear alongside IPF, says Dr. Ng; when that is the case, they are addressed as well.
Your takeaway? If you have a persistent cough or changes in your breathing without a clear cause, see your doctor and follow up. Please visit beyondthescars.com to learn more about interstitial lung disease and how to manage the condition.
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