What is non-specific interstitial pneumonia? | Action for Pulmonary Fibrosis

Non-specific interstitial pneumonia (NSIP) patients are usually younger that people with IPF, and more likely to be female.

Causes of non-specific interstitial pneumonia

Non-specific interstitial pneumonia can be associated with autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus (SLE), Sjogren’s syndrome and rheumatoid arthritis.

It can also be idiopathic, which means no specific cause can be identified.

Symptoms

People with NSIP are usually breathless and may have a cough. They may also experience other symptoms of autoimmune diseases such as skin rashes, joint pains, dry eyes, dry mouth, mouth ulcers and fatigue. Find out more about pulmonary fibrosis and its symptoms.

Treatments

Typically, you will be offered a trial of immunosuppressive therapy, particularly if the NSIP is associated with an autoimmune disease.

You may also be offered pulmonary rehabilitation and oxygen therapy to help improve your quality of life.

Ongoing treatment

Your medical team will monitor your lung disease regularly to determine if your treatment is effective.

Outlook

In general, the life expectancy for NSIP is better than for people with idiopathic pulmonary fibrosis. Patients usually survive for five years after diagnosis, with 26-40% living for more than ten years. However, life expectancy is determined by rate of disease progression and response to treatment.

Other forms of pulmonary fibrosis

Get support

Join a support group in your area

It’s important to remember that you are not alone. There are now over 75 pulmonary fibrosis support groups around the country.

Support groups meet informally to share experiences, provide expert information and raise funds and awareness. Find a group near you.

Call our support line

APF runs an email and telephone support line offering information and advice about living with PF. Find out more.

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Watch videos of how other people cope day to day with PF.

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Information provided by Action for Pulmonary Fibrosis (APF) is not a substitute for professional medical advice. It’s intended as general information only. APF is not responsible for any errors or omissions or for any loss or damage suffered by users resulting from the information published on actionpf.org.