Patient Information for Non-specific interstitial ...



Patient Information for Non-specific interstitial pneumonitis (NSIP)

What is NSIP?

NSIP is the name given to a collection of lung diseases that can cause progressive scarring of the lungs if left untreated. NSIP affects the area of the lungs where oxygen is absorbed from the air. The scar tissue prevents the lungs from working normally. The amount of scarring progresses with time and is generally irreversible. Some people have significant inflammation with this condition, which if found and treated early, is usually reversible.

Who develops NSIP?

Anyone can develop NSIP and it can affect anyone of any age. As yet the cause is unknown. The symptoms can appear similar to another lung disease called Idiopathic Pulmonary Fibrosis (IPF). However, it can be more responsive to treatment than IPF if diagnosed and treated early.

What are the symptoms of NSIP?

The main symptom is usually shortness of breath on exertion, for example walking up hills or stairs. Some people describe having a cough which can be dry or they might produce thick white or clear phlegm.

How is NSIP diagnosed?

Diagnosis is usually made by a specialist and therefore patients will be referred by their GP to a hospital for the following investigations.

• A chest x-ray. This may show signs of scarring and inflammation

• A CT scan. This is a special x-ray technique which gives a more detailed picture of your lungs. There are characteristic features on the CT scan which will help your doctor to make a diagnosis.

• Lung function tests. These are breathing tests that show how well your lungs are working. They are used regularly to monitor your progress and response to treatment.

• Blood tests. These are usually done to determine your general health and to exclude any other lung diseases.

• Bronchoscopy. Some patients may have a camera test whereby a flexible telescope is passed down into the lungs to collect samples.

• Surgical lung biopsy. This happens under general anaesthetic to remove small pieces of lung tissue to sometimes help with the diagnosis.

How is NSIP treated?

Treatment aims to reduce any inflammation but it is impossible to reverse any scarring. Steroids are used in the first instance, and often have an immediate effect. Other drugs are soon added that work with the steroids but do not have the same side-effects. Steroids can cause weight gain, thinning of the bones and diabetes. Together these drugs may depress your immune system making you more susceptible to infections. You will need careful monitoring with regular blood tests. It is important that you have regular flu and pneumonia vaccinations when on immune depressant drugs.

Sometimes it may be necessary to have oxygen therapy to relieve breathlessness. Your clinician will be able to advise you about this.

Further information

This sheet contains general information about NSIP. As the disease affects different people in different ways, it is best to discuss your condition with the staff who are caring for you.

Further information is available from:

• North West Lung Centre, Wythenshawe Hospital 0161-2914936

• Wythenshawe hospital medicines information helpline on 0161 2913331

• nhsdirect.nhs.uk

• patient.co.uk

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