Pulmonary Fibrosis: Symptoms, Causes, Diagnosis and Treatment

Pulmonary fibrosis is not one single disease. It describes scarring within lung tissue that makes the lungs stiffer and interferes with the efficient transfer of oxygen into the bloodstream. Fibrosis can occur within many different interstitial lung diseases, some with identifiable causes and others without a known cause.[1]

Therefore, seeing the word fibrosis on a CT report does not automatically mean that a person has Idiopathic Pulmonary Fibrosis (IPF), nor does a pulmonary fibrosis diagnosis mean that every patient will follow the same clinical course.

What Is Pulmonary Fibrosis?

Pulmonary fibrosis refers to scar tissue developing within the lungs. Fibrotic tissue makes the lungs stiffer and interferes with oxygen moving from the air sacs into the bloodstream, which can lead to breathlessness, particularly with physical activity.[1]

The term alone does not identify the cause, expected rate of progression, or appropriate treatment.

Is Pulmonary Fibrosis One Disease?

No.

The Pulmonary Fibrosis Foundation describes pulmonary fibrosis as a family of more than 200 lung diseases with overlapping features but different causes, treatments, and clinical courses.[1]

Many fall within the broader category of interstitial lung diseases (ILDs).

What Is the Difference Between PF, IPF and PPF?

IPF is therefore not another name for all pulmonary fibrosis, and PPF describes progression across multiple non-IPF fibrotic ILDs rather than one single disease.[5]

What Are the Symptoms of Pulmonary Fibrosis?

Common symptoms include:

• Shortness of breath, initially often during exertion.
• Persistent dry cough.
• Fatigue and weakness.
• Reduced ability to perform usual activities.
• Loss of appetite or unexplained weight loss in some people.
• Chest discomfort in some patients.

Some patients also develop finger clubbing, although clubbing is not specific to pulmonary fibrosis and can occur with other lung, heart, and liver conditions.[3]

What Are the Early Symptoms of Pulmonary Fibrosis?

Early disease may cause very mild symptoms or no obvious symptoms at all.

A person may gradually notice that they:

• Become breathless sooner while walking.
• Need to stop while climbing stairs.
• Can no longer perform familiar activities as comfortably.
• Develop a persistent dry cough without an obvious explanation.

As fibrosis progresses, breathlessness can begin to affect increasingly routine daily activities.[3]

What Causes Pulmonary Fibrosis?

Several major groups of causes are recognized.[2]

Does Smoking Cause Pulmonary Fibrosis?

Smoking is associated with increased risk of some interstitial lung diseases and is a recognized risk factor for IPF, but it is not the explanation for every case of fibrosis.

A person who smokes and has fibrotic changes still requires evaluation for other relevant causes.

Is Pulmonary Fibrosis Contagious?

No. Fibrosis itself is not an infection and cannot spread between people.

Some infections can cause lung injury that later leaves scarring, but the resulting fibrosis is not contagious.

How Is Pulmonary Fibrosis Diagnosed?

Diagnosis usually requires several pieces of information rather than one test.

The assessment may include:

• Medical and family history.
• Smoking history.
• Occupational exposures.
• Bird, mold, and other environmental exposures.
• Medication history.
• Symptoms or history suggesting autoimmune disease.
• High-resolution CT.
• Pulmonary function tests.
• Oxygen assessment at rest and during exertion.
• Blood tests when appropriate.
• Bronchoscopy or sampling in selected patients.
• Lung biopsy in a smaller number of cases when the diagnosis remains uncertain.

The Pulmonary Fibrosis Foundation notes that some patients remain without a definitive diagnosis even after extensive testing, and lung biopsy may occasionally be needed to clarify the type of disease.[4]

Why Is HRCT Important?

High-resolution computed tomography provides detailed images of lung tissue and allows subtle patterns of inflammation and fibrosis to be evaluated.

These details can be critical because several forms of pulmonary fibrosis may appear broadly similar while showing different characteristic patterns on HRCT.[4]

However, fibrotic change on CT is not automatically equivalent to IPF. Imaging findings must be interpreted in clinical context.

Diffuse lung fibrosis is also different from a focal pulmonary nodule. If your report describes a nodule or spot rather than diffuse fibrosis, see Lung Nodules: Does Every Spot Mean Cancer?.

What Are FVC and DLCO?

Pulmonary function tests measure how well the lungs work rather than what they look like.

• FVC – Forced Vital Capacity: the amount of air that can be forcefully exhaled after taking a full breath.
• DLCO: assesses how effectively gas moves from the lungs into the bloodstream.

Trends over time can be more informative than one isolated measurement when determining whether fibrotic lung disease is stable or progressing.[5]

How Does a Doctor Know if Pulmonary Fibrosis Is Progressing?

Some fibrotic ILDs develop a disease behavior called Progressive Pulmonary Fibrosis (PPF).

According to the ATS/ERS/JRS/ALAT guideline, PPF in a patient with fibrotic ILD other than IPF is defined by at least two of three features occurring within one year, with no alternative explanation:

• Worsening respiratory symptoms.
• Physiological evidence of progression.
• Radiological progression.[5]

Physiological progression can include an absolute decline in predicted FVC of at least 5%, or an absolute decline in predicted DLCO of at least 10% within a year when attributable to worsening fibrosis.[5]

Can Pulmonary Fibrosis Be Cured?

The answer depends on the specific form of disease and what is meant by “cure.”

Established scar tissue generally cannot be converted back into normal lung tissue.

However, treatment can still be meaningful. In some forms of ILD, clinicians can remove or treat the underlying trigger, control autoimmune or inflammatory disease, slow further fibrosis, manage symptoms, and preserve function for as long as possible.

Even in IPF, where no treatment currently reverses established fibrosis, available therapies can slow lung-function decline in appropriate patients.[6]

How Is Pulmonary Fibrosis Treated?

Treatment depends on the cause and type of ILD.

A management plan may include:

• Removing a relevant environmental or occupational exposure.
• Reviewing and stopping a causative medication under medical supervision when appropriate.
• Treating associated autoimmune or inflammatory disease.
• Antifibrotic therapy in selected forms of disease.
• Supplemental oxygen when clinically indicated.
• Pulmonary rehabilitation.
• Smoking cessation.
• Vaccination and infection prevention according to medical advice.
• Management of symptoms and complications.
• Lung-transplant evaluation in selected patients.

Can Medication Slow Pulmonary Fibrosis?

Yes. Antifibrotic therapies can slow lung-function decline in selected fibrotic lung diseases.

Internationally established therapies for IPF include nintedanib and pirfenidone, depending on diagnosis, clinical suitability, and local regulatory approval.[6]

The treatment landscape has also changed recently. In the United States, the FDA approved nerandomilast for adults with IPF in October 2025 and extended its approval to adults with Progressive Pulmonary Fibrosis in December 2025.[7]

Regulatory approval and availability vary by country, and no antifibrotic is appropriate for every patient.

These therapies are intended to slow disease progression; they do not remove existing scar tissue.

Do Corticosteroids Treat Pulmonary Fibrosis?

Not as a universal treatment.

Corticosteroids or other immune-modifying treatments may be appropriate in some inflammatory or autoimmune ILDs.

The Pulmonary Fibrosis Foundation notes that prednisone is not usually used to treat IPF, although it can be used in other forms of pulmonary fibrosis where inflammation is clinically important.[6]

What Is the Role of Oxygen and Pulmonary Rehabilitation?

Supplemental oxygen may be prescribed when blood oxygen falls below appropriate levels at rest or during activity.

Pulmonary rehabilitation is a structured program that can include:

• Exercise training.
• Breathing techniques.
• Disease education.
• Strategies for managing breathlessness.
• Nutritional and psychological support where available.

Pulmonary rehabilitation can improve physical function, breathlessness, mood, and quality of life in people with IPF and other forms of pulmonary fibrosis.[8]

When Is Lung Transplantation Considered?

Lung transplantation is not required for everyone with pulmonary fibrosis, but it can be considered for selected patients with advanced disease who meet transplant criteria.

Early referral can be important in progressive disease because transplant assessment itself is a detailed process involving overall health, organ function, age, surgical risk, and other factors.[9]

Is Pulmonary Fibrosis Dangerous, and What Determines Prognosis?

Pulmonary fibrosis can be serious, but there is no single disease course or life-expectancy figure that applies to every patient.

Factors that can affect prognosis include:

• The specific ILD diagnosis.
• The cause of fibrosis.
• HRCT pattern and disease extent.
• FVC and DLCO and how they change over time.
• Oxygen requirements.
• Rate of symptom progression.
• Pulmonary hypertension.
• Acute exacerbations.
• Age and other medical conditions.
• Response to treatment.

Advanced fibrotic lung disease can be associated with complications such as hypoxemia, pulmonary hypertension, respiratory failure, and acute exacerbations.[10]

When Does Pulmonary Fibrosis Require Urgent Assessment?

Urgent medical assessment is appropriate when there is significant or sudden deterioration such as:

• Sudden or severe worsening of breathlessness.
• A substantial drop in oxygen compared with usual levels.
• New or severe chest pain.
• Blue lips or extremities.
• Confusion or loss of consciousness.
• Fever or signs of infection with worsening breathing.
• Coughing up blood.

A sudden deterioration does not automatically mean that fibrosis itself has progressed. Infection, pulmonary embolism, heart disease, acute exacerbation, and other causes may need to be considered.

Pulmonary Fibrosis Diagnosis and Follow-Up at Mouwasat Hospital

The Pulmonology Department at Mouwasat Hospital lists diagnosis and management of interstitial lung diseases including idiopathic pulmonary fibrosis, sarcoidosis, hypersensitivity pneumonitis, and connective-tissue-related ILD, together with high-resolution CT and multidisciplinary collaboration when appropriate.[11]

Published pulmonology physician profiles at Mouwasat also include experience in pulmonary fibrosis, ILD, and pulmonary function testing, allowing follow-up to incorporate symptoms, imaging, and lung-function trends rather than relying on a single result.[12]

If persistent cough, weight loss, or other symptoms have raised concerns about a different lung condition, see our Lung Cancer Symptoms and Risk Factors Guide. Symptoms alone cannot diagnose either condition.

Frequently Asked Questions About Pulmonary Fibrosis

Can pulmonary fibrosis be cured?

Established fibrosis generally cannot be returned to normal lung tissue. However, some causes can be treated or removed, and therapies may slow disease progression and improve symptoms and function.

Is every case of pulmonary fibrosis IPF?

No. IPF is one specific form of pulmonary fibrosis diagnosed according to defined criteria when no appropriate underlying cause is identified.

Can pulmonary fibrosis be fatal?

Some forms can progress and lead to serious complications, but disease course varies greatly and cannot be predicted from the general diagnosis or a CT report alone.

Is pulmonary fibrosis contagious?

No. Pulmonary fibrosis itself cannot spread from person to person.

Is pulmonary fibrosis cancer?

No. Fibrosis is scar formation, whereas cancer involves abnormal cell growth. They are different diseases requiring different evaluation and treatment.

Can pulmonary fibrosis be seen on CT?

Yes. CT can show fibrotic abnormalities, and HRCT provides greater detail for characterizing their pattern and distribution. Imaging still needs to be interpreted alongside other clinical information.

What does “mild pulmonary fibrosis” mean?

It may describe limited changes on imaging, but the phrase alone does not establish the cause or future progression. Pattern, symptoms, lung function, and follow-up all matter.

Can someone live for years with pulmonary fibrosis?

Yes. The course varies widely among diseases and individuals, and some conditions can remain stable or progress slowly for long periods.

Is exercise safe with pulmonary fibrosis?

Appropriate activity is often beneficial and forms part of pulmonary rehabilitation, but exercise intensity and oxygen requirements should be individualized.

Does oxygen treat the fibrosis itself?

No. Supplemental oxygen treats low blood oxygen and can improve activity tolerance in appropriate patients, but it does not remove lung scarring.

Do steroids treat IPF?

Corticosteroids are not routine treatment for stable IPF. They may have a role in other inflammatory or autoimmune forms of ILD.

Can pulmonary fibrosis stop progressing?

Some fibrotic diseases remain stable for periods of time, while others progress. Serial assessment of symptoms, pulmonary function, and imaging helps determine each patient's disease behavior.

Final Thoughts: Identifying the Type of Fibrosis Matters More Than the Word “Fibrosis” Alone

Pulmonary fibrosis is not one diagnosis and does not automatically mean IPF or rapidly progressive disease.

The key questions are:

• Which interstitial lung disease is present?
• Is there an identifiable cause or exposure?
• What pattern is present on HRCT?
• What do FVC and DLCO show?
• Is the disease stable or progressing?

Those answers guide whether management should focus on removing a trigger, controlling inflammation or autoimmune disease, antifibrotic treatment, oxygen and rehabilitation, or advanced options such as lung-transplant evaluation in selected patients.

Medical disclaimer: This article is intended for general health education and does not replace assessment by a pulmonologist. Do not start or stop medication based on this information.

Medical References

1. Pulmonary Fibrosis Foundation — What Is Pulmonary Fibrosis?
2. Pulmonary Fibrosis Foundation — Causes
3. Pulmonary Fibrosis Foundation — Symptoms
4. Pulmonary Fibrosis Foundation — Diagnosis
5. ATS/ERS/JRS/ALAT — IPF Update and Progressive Pulmonary Fibrosis Guideline
6. Pulmonary Fibrosis Foundation — Medications
7. U.S. FDA — Nerandomilast for Progressive Pulmonary Fibrosis
8. Pulmonary Fibrosis Foundation — Pulmonary Rehabilitation
9. Pulmonary Fibrosis Foundation — Progressive Pulmonary Fibrosis
10. NHLBI — Idiopathic Pulmonary Fibrosis
11. Mouwasat Hospital — Pulmonology
12. Mouwasat Hospital — Pulmonology and Interstitial Lung Diseases