Nonspecific interstitial pneumonia (NSIP) is a type of interstitial lung disease that causes inflammation between the air sacs of your lungs. It causes a cough and shortness of breath that get worse over time. It’s often associated with connective tissue disorders. If you don’t have scarring in your lungs, NSIP is usually curable with treatment.
Nonspecific interstitial pneumonia (NSIP) is inflammation in the spaces between the air sacs in your lungs (alveoli) and the blood vessels around them. It’s a type of interstitial lung disease. It most commonly affects people with connective tissue disease (CTD), but it’s also associated with other conditions.
Unlike other types of pneumonia, nonspecific interstitial pneumonia can last a long time and sometimes causes permanent scarring in your lungs.
Providers categorize NSIP as either cellular or fibrotic:
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Usual interstitial pneumonia is another name for idiopathic pulmonary fibrosis (IPF), the most common interstitial lung disease. IPF causes irreversible lung scarring. NSIP doesn’t always cause lung scarring and can often be cured.
How serious interstitial pneumonia is depends on whether there’s damage to your lungs and, if so, how bad the damage is. Usual interstitial pneumonia and fibrotic NSIP have a worse prognosis than cellular NSIP.
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Symptoms of nonspecific interstitial pneumonia might be subtle at first and get worse over time. They include:
Experts aren’t sure what causes nonspecific interstitial pneumonia. People with NSIP tend to have other conditions or diseases, but there isn’t strong evidence that those conditions cause NSIP. For instance, connective tissue diseases (those that affect the structural tissues of your body, including autoimmune disorders) are often associated with NSIP, but they don’t necessarily cause NSIP. Another cause may lead to both CTD and NSIP.
NSIP is most commonly diagnosed in women and people assigned female at birth (AFAB) between the ages of 40 and 50. Other conditions and diseases associated with NSIP include:
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A healthcare provider diagnoses nonspecific interstitial pneumonia based on your symptoms and what your lungs look like on imaging. Tell your provider about any other health conditions you have or medications you’re taking.
Specific tests and procedures you might need include:
Providers treat nonspecific interstitial pneumonia with medications that reduce inflammation and manage underlying conditions. In severe cases, treatments like pulmonary rehabilitation and oxygen therapy can strengthen your lungs and help you get more oxygen. If your condition worsens despite treatment, a lung transplant may be an option.
Specific medications and procedures to treat NSIP include:
Since we’re unsure of the exact causes of NSIP, there’s no specific way to prevent it. Managing underlying conditions, especially connective tissue disorders, might reduce your risk.
The prognosis for people with cellular NSIP is good — the five-year survival rate is 100%. You may need to take medications and follow up with your healthcare provider for several months to heal.
Fibrotic NSIP causes irreversible damage to your lungs that can get worse over time. Most people with fibrotic NSIP live six to 14 years after diagnosis.
If you’ve been diagnosed with NSIP, make a plan with your healthcare provider to manage any underlying conditions and take all medications as prescribed. This can prevent irreversible damage to your lungs. Let your provider know if you have any new symptoms or your symptoms get worse.
See your healthcare provider if you’ve had increasing shortness of breath, shortness of breath with exercise or an ongoing dry cough. NSID is more likely to get progressively worse if left untreated.
It might be helpful to ask your healthcare provider:
Pneumonia inflames your alveoli. They sometimes fill with fluid or pus. Interstitial pneumonia causes inflammation in the structural spaces of your lungs, in between your alveoli. Pneumonia is much more common and is usually caused by viruses or bacteria. The cause of NSIP often isn’t known.
A note from Cleveland Clinic
Many conditions can cause a cough and make you tired when you exert yourself. But if your symptoms have gone on for several weeks or have been getting worse over time, it’s important to see a healthcare provider. Many people with NSIP get better with treatment and avoid permanent lung damage.
Last reviewed on 09/28/2023.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy