Nonspecific Interstitial Pneumonia (NSIP)

What is nonspecific interstitial pneumonia (NSIP)?

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.

Types of NSIP

Providers categorize NSIP as either cellular or fibrotic:

• In cellular NSIP, the cells between your alveoli are inflamed.
• In fibrotic NSIP, your lung tissue is thick and scarred. This is a more serious form of NSIP.

What’s the difference between usual interstitial pneumonia and nonspecific interstitial pneumonia?

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 is interstitial pneumonia?

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.

What are the symptoms of nonspecific interstitial pneumonia (NSIP)?

Symptoms of nonspecific interstitial pneumonia might be subtle at first and get worse over time. They include:

• A dry cough.
• Shortness of breath.
• Difficult or labored breathing.
• Fatigue.
• Clubbed fingers (uncommon).

What causes nonspecific interstitial pneumonia (NSIP)?

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.

Risk factors for 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:

• Connective tissue disorders like rheumatoid arthritis, lupus and scleroderma.
• Viral infections, including HIV and hepatitis.
• Reactions to certain medications, like cancer chemotherapy, radiation therapy and certain heart medications (drug-induced interstitial pneumonia).

How is NSIP diagnosed?

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.

Tests and procedures

Specific tests and procedures you might need include:

• Pulmonary (lung) function tests.
• Chest X-ray.
• CT scan (computed tomography scan).
• Bronchoscopy.
• Biopsy.

How is nonspecific interstitial pneumonia treated?

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.

NSIP medications and procedures

Specific medications and procedures to treat NSIP include:

• Prednisone or other corticosteroids.
• Immunosuppressive and cytotoxic drugs, including azathioprine, cyclophosphamide and mycophenolate.
• Anti-fibrotic drugs, including pirfenidone and nintedanib.
• Rituximab, a biologic drug for connective tissue disorders.
• Pulmonary rehabilitation.
• Oxygen therapy.
• Lung transplant.

Can NSIP be prevented?

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.

What’s the outlook for NSIP?

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.

How do I take care of myself?

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.

When should I see my healthcare provider?

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.

What questions should I ask my doctor?

It might be helpful to ask your healthcare provider:

• What’s causing this?
• What are my treatment options?
• Will it get worse?
• Can the damage be slowed down?
• What can I do to take care of myself?
• When should I follow up with you?

What is the difference between pneumonia and interstitial pneumonia?

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.