Understanding Nonspecific Interstitial Pneumonia (NSIP): Symptoms and Recovery
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: Key Facts About NSIP
Nonspecific interstitial pneumonia (NSIP) is a type of interstitial lung disease that causes a dry cough, worsening shortness of breath and tiredness. [1] Its cause is often unknown but frequently linked to connective tissue diseases such as rheumatoid arthritis and lupus. [1] Cellular NSIP has about 100% five-year survival, while fibrotic NSIP is more serious. [1] Treatments include corticosteroids and immune-suppressing drugs. [1] [2]
Feature | Details |
Condition type | A type of interstitial lung disease (ILD). |
Main symptoms | A dry cough, shortness of breath that gets worse over time, and tiredness. |
Main causes | Often unknown. Frequently linked to connective tissue diseases such as rheumatoid arthritis, lupus, and scleroderma. |
Two types | Cellular (inflammation) and fibrotic (scarring). |
Outlook | Cellular NSIP: five-year survival is about 100%. Fibrotic NSIP: most people live 6 to 14 years after diagnosis. [1] |
Core treatments | Corticosteroids, immune-suppressing drugs, anti-fibrotic drugs, and supportive care such as oxygen and pulmonary rehabilitation. |
What the research says
The outlook for idiopathic NSIP is generally better than for IPF. A 2025 Korean clinical guideline reports that many studies find a 5-year survival rate above 70% for idiopathic NSIP. The guideline notes that treatment often uses steroids alone or with other immune-suppressing drugs, but evidence that these treatments work is limited. It advises considering antifibrotic drugs when the scarring keeps getting worse (progressive pulmonary fibrosis). (Jo et al., Tuberc Respir Dis, 2025) [2]
Nintedanib slowed lung function loss in progressive scarring lung disease. In 663 people with progressive fibrosing interstitial lung disease, lung capacity (FVC) fell by 80.8 mL per year with nintedanib versus 187.8 mL per year with placebo, a difference of 107.0 mL per year. Diarrhea was the most common side effect (66.9% versus 23.9%). (Flaherty et al., N Engl J Med, 2019) [3]
Mycophenolate was better tolerated than cyclophosphamide in scleroderma-related lung disease. In 142 people with scleroderma-related interstitial lung disease, lung function (% predicted FVC) improved with both drugs over 24 months (2.19 points with mycophenolate and 2.88 with cyclophosphamide), with no significant difference between them. Low white blood cell counts were more common with cyclophosphamide (30 people versus 4). (Tashkin et al., Lancet Respir Med, 2016) [4]
What Is Nonspecific Interstitial Pneumonia (NSIP)?
Nonspecific interstitial pneumonia (NSIP) is inflammation in the spaces between the air sacs in your lungs (the alveoli) and the blood vessels around them. [1] It is a type of interstitial lung disease. [1] Unlike common pneumonia, which is usually caused by bacteria or viruses and often fills the air sacs with fluid or pus, NSIP affects the supporting tissue between the air sacs. [1]
NSIP most commonly affects people with connective tissue disease, although it is also linked to other conditions. [1] When NSIP shows up, it can be a clue that something else is going on in the body, such as an autoimmune condition. How serious it is depends on whether the lung tissue is only inflamed or has started to scar.
What are the two types of nonspecific interstitial pneumonia (NSIP)?
Doctors divide NSIP into two types, based on how the lung tissue looks under the microscope. Telling them apart matters because it guides treatment and shapes the long-term outlook.
Cellular NSIP: the cells between the air sacs are inflamed. [1] This is the more favorable type, because inflammation can often be reversed with medicine before it turns into permanent damage.
Fibrotic NSIP: the lung tissue has become thick and scarred. This is the more serious type. [1] The scarring is permanent and can get worse over time [1], which makes it harder to manage.
What are the symptoms and warning signs of NSIP?
NSIP symptoms are often subtle at first, so people mistake them for a lingering cold or for getting older. They can get worse over time. [1]
A dry cough [1]: a cough that does not bring up mucus and does not improve with over-the-counter remedies.
Shortness of breath that gets worse [1]: at first you may notice it only with effort. Over time, breathing can feel hard or labored. [1]
Fatigue [1]: a deep tiredness that sleep does not fix, often because breathing takes extra effort.
Clubbed fingers (uncommon) [1]: the tips of the fingers or toes become wider and rounder, which can happen when oxygen levels stay low for a long time.
What causes nonspecific interstitial pneumonia and who is at risk?
Experts are not sure what causes NSIP. [1] People with NSIP often have other conditions, but there is no strong evidence that those conditions directly cause it. For example, connective tissue diseases are often linked to NSIP, but another underlying cause may lead to both. [1] NSIP is most commonly diagnosed in women between the ages of 40 and 50. [1]
Category | Linked conditions or triggers |
Connective tissue (autoimmune) diseases | Rheumatoid arthritis, lupus (systemic lupus erythematosus), and scleroderma. [1] |
Viral infections | Viral infections, including HIV and hepatitis. [1] |
Medicine and treatment reactions | Reactions to certain medicines, such as cancer chemotherapy and certain heart medicines, and to radiation therapy (drug-induced interstitial pneumonia). [1] |
How Is NSIP Diagnosed?
Diagnosing NSIP takes teamwork, because its symptoms overlap with many other lung conditions. A healthcare professional diagnoses it based on your symptoms and how your lungs look on imaging. [1] Tell them about any other health conditions you have and all medicines you take.
Pulmonary (lung) function tests measure how much air your lungs hold and how well they move oxygen into your blood. A chest X-ray and a CT scan, especially a high-resolution CT, show patterns of inflammation or scarring in the lung tissue. If imaging does not give a clear answer, a bronchoscopy or a lung biopsy may be needed so a small piece of tissue can be examined. [1]
How is nonspecific interstitial pneumonia (NSIP) treated?
The goal of treatment is to calm lung inflammation. [1] Treatment also focuses on any underlying condition. Cellular NSIP is usually curable with treatment. [1]
Anti-inflammatory therapy: corticosteroids such as prednisone are the usual first treatment. [1] [2] They reduce inflammation in lung tissue. [1]
Immune-suppressing drugs: your doctor may add drugs such as azathioprine, mycophenolate, or cyclophosphamide. [1] [2] Rituximab, a biologic drug, may be used for connective tissue disorders. [1]
Anti-fibrotic drugs: in fibrotic NSIP, pirfenidone or nintedanib may be used to slow scarring. [1] [3]
Supportive care: in severe cases, pulmonary rehabilitation and oxygen therapy strengthen the lungs and help you get more oxygen. [1] If the condition keeps worsening despite treatment, a lung transplant may be an option. [1]
What is the outlook for NSIP, and how can people live well with it?
The outlook for cellular NSIP is good. The five-year survival rate is about 100%, though you may need to take medicines and see your healthcare professional for several months while you heal. [1] Fibrotic NSIP causes irreversible lung damage that can get worse over time, and most people with it live six to 14 years after diagnosis. [1]
If you are diagnosed with NSIP, work with your healthcare professional on a plan to manage any underlying conditions and take every medicine as prescribed. This helps prevent irreversible lung damage. Report new symptoms, or symptoms that get worse, right away. [1] NSIP is more likely to keep getting worse if it is left untreated. [1]
What is the bottom line on nonspecific interstitial pneumonia?
Nonspecific interstitial pneumonia is a complex condition, and early treatment gives the best outcome. Fibrotic NSIP is a serious diagnosis, but cellular NSIP has a high chance of full recovery with proper care. If you have a dry cough that will not go away or shortness of breath that keeps increasing, seeing a healthcare professional, ideally a lung specialist (pulmonologist), is the most important step toward protecting your lungs.
Short of breath, or coughing for weeks? Don't wait it out. A healthcare professional can order lung function tests and imaging to find the cause and start treatment early.
Frequently Asked Questions
Is NSIP the same as regular pneumonia?
No. Regular pneumonia is usually an infection that inflames the air sacs. NSIP is a long-lasting inflammatory condition of the supporting spaces between the air sacs, and its cause often is not known. [1]
Can NSIP be cured?
Cellular NSIP is usually curable with treatment. [1] Fibrotic NSIP causes irreversible scarring, so it is not usually curable, but treatment can help slow it down. [3]
What is the survival rate for NSIP?
For cellular NSIP, the five-year survival rate is about 100%. [1] Most people with fibrotic NSIP live between 6 and 14 years after diagnosis. [1]
Does NSIP always lead to lung scarring?
No. Only fibrotic NSIP involves scarring. [1] In cellular NSIP, the inflammation can often be treated before scarring develops.
Who is most at risk for developing NSIP?
NSIP is most commonly diagnosed in women between 40 and 50. [1] People with connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma are also affected more often. [1]
Can smoking cause NSIP?
Smoking is a major risk factor for many lung diseases, but it is not among the conditions and exposures linked to NSIP in Cleveland Clinic's overview. Quitting smoking still protects your lungs.
Is NSIP contagious?
No. NSIP is an inflammatory condition, often linked to connective tissue disease, and it cannot be spread from person to person.
How is NSIP different from pulmonary fibrosis (IPF)?
IPF is another name for usual interstitial pneumonia. It always causes irreversible lung scarring and has a worse outlook. NSIP does not always cause scarring and can often be cured. [1]
What medicines are used for NSIP?
Common treatments include prednisone and other corticosteroids, immune-suppressing drugs such as azathioprine, mycophenolate, and cyclophosphamide, rituximab for connective tissue disorders, and anti-fibrotic drugs such as pirfenidone and nintedanib. [1]
Will I need oxygen therapy?
Oxygen therapy and pulmonary rehabilitation are used in severe cases, when the lungs cannot get enough oxygen into the blood on their own. [1]
Can I exercise with NSIP?
Pulmonary rehabilitation, a supervised program of exercise and breathing training, can help strengthen your lungs. [1] Ask your healthcare professional what level of activity is safe for you.
Is a lung transplant necessary for NSIP?
Not for most people. A lung transplant may be an option if your condition keeps getting worse despite treatment. [1]
How often will I need follow-up visits?
It varies. People with cellular NSIP may need medicines and follow-up visits for several months. [1] Your healthcare professional will set a schedule that fits your situation.
Are there natural remedies for NSIP?
There are no proven natural cures for NSIP. Taking your medicines as prescribed, managing any underlying condition, and keeping your follow-up visits are what protect your lungs. Ask your healthcare professional before taking any supplement.
Can viral infections cause NSIP?
Certain viral infections, including HIV and hepatitis, are linked to NSIP. [1] Experts do not know whether they cause it.
Related Reading
Interstitial Lung Disease: What It Is, What Causes the Scarring, and What Treatment Can Actually Do explains the lung disease group that includes NSIP.
Pulmonary Fibrosis: Symptoms, Causes, Diagnosis, and Treatment Explained explains lung scarring, its symptoms and treatment options.
High-Resolution Computed Tomography (HRCT): A Detailed Guide to Advanced Lung Imaging describes the detailed lung CT scan used to spot NSIP.
Lung Biopsy: Types, What to Expect, Recovery, Risks and Results explains how lung tissue samples are taken and tested.
References
Cleveland Clinic. Nonspecific interstitial pneumonia (NSIP). Cleveland Clinic. Last updated September 28, 2023. https://my.clevelandclinic.org/health/diseases/14804-nonspecific-interstitial-pneumonia-nsip
Jo YS, Lee HK, Park SH, et al. Korean Guidelines for Diagnosis and Management of Idiopathic Nonspecific Interstitial Pneumonia. Tuberc Respir Dis (Seoul). 2025;88(2):237-246. PMID 39761948. https://doi.org/10.4046/trd.2024.0168
Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019;381(18):1718-1727. PMID 31566307. https://doi.org/10.1056/NEJMoa1908681
Tashkin DP, Roth MD, Clements PJ, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708-719. PMID 27469583. https://doi.org/10.1016/S2213-2600(16)30152-7
Medical disclaimer
This article is for general informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional for diagnosis and treatment of any lung condition.
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