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Rare lung & airway condition

Non-specific interstitial pneumonia (NSIP)

Non-specific interstitial pneumonia is a pattern of lung inflammation and fibrosis with a generally better outlook than IPF; it is often linked to autoimmune disease or drugs, and frequently responds to immunosuppression.

Key points

The essentials at a glance.

What to look out for, how the diagnosis is confirmed, who looks after it, and the options that exist.

  • 01

    NSIP is a pattern seen on CT and biopsy, not a single disease - an underlying cause, especially connective tissue disease, is always sought.

  • 02

    The cellular (inflammatory) form responds best to treatment; the fibrotic form is more stubborn but still generally outperforms IPF.

  • 03

    Diagnosis is agreed at a specialist ILD MDT combining imaging, immunology and sometimes biopsy.

  • 04

    First-line treatment is usually corticosteroids with a steroid-sparing agent such as mycophenolate.

  • 05

    Progressive fibrotic NSIP despite treatment may qualify for antifibrotic therapy.

Understanding it

What it is, and what happens next.

In plain English - what is going on, how specialists confirm it, and the routes to treatment in the UK.

NSIP describes a uniform, temporally homogeneous pattern of inflammation and fine fibrosis spread through the lung - a contrast to the patchy, honeycombed geography of IPF, and a distinction with real consequences, since NSIP frequently signals an underlying autoimmune process and answers to immunosuppression in a way IPF does not.

Because so much rides on the pattern, cases deserve high-resolution CT reviewed at an ILD MDT, autoimmune serology, and a careful drug and exposure history; treatment then typically runs steroids into mycophenolate with lung function tracked over months, escalating or adding nintedanib for the minority whose fibrosis advances regardless. In many parts of the UK, routine NHS outpatient referrals for problems like this can take several months, whereas an initial appointment with a private consultant is typically available within around two weeks.

A quiet reminder

This guide is for information, not medical advice.

Your GP or specialist knows your history and can tell you which parts apply to you. If in doubt, speak to them.

Published 2026-09-27 · Next review 2027-09-27

Frequently asked

Common questions about Non-specific interstitial pneumonia (NSIP).

Quick, plain-English answers to what people ask us most.

  • What is non-specific interstitial pneumonia (NSIP)?

    NSIP is a type of interstitial lung disease causing inflammation, and sometimes scarring, of the lung tissue between the air sacs, occurring either alone or associated with an underlying autoimmune connective tissue disease.

  • What causes NSIP?

    NSIP is often linked to autoimmune connective tissue diseases such as scleroderma, rheumatoid arthritis or myositis, though it can occur without an identifiable underlying cause, known as idiopathic NSIP.

  • What are the symptoms of NSIP?

    Symptoms include progressive breathlessness, a persistent dry cough, and fatigue, developing gradually over months, sometimes alongside symptoms of an associated connective tissue disease.

  • Is NSIP dangerous?

    NSIP generally has a more favourable outlook than some other interstitial lung diseases, particularly the more inflammatory cellular subtype, though it can still cause significant breathlessness and progressive lung changes.

  • Can NSIP be treated?

    Yes, NSIP often responds to immunosuppressive treatment, particularly corticosteroids, especially the cellular subtype, with treatment also addressing any underlying connective tissue disease if present.

  • How is NSIP diagnosed?

    Diagnosis typically involves high-resolution CT imaging of the chest, lung function testing, and sometimes a lung biopsy, alongside blood tests to check for an associated connective tissue disease.

  • When should I see a respiratory specialist about suspected NSIP, and how quickly can I be seen privately?

    See a respiratory specialist for progressive breathlessness with a dry cough. A private respiratory physician can typically arrange assessment within about two weeks.