Pulmonary Fibrosis - Diagnosis and Treatment

Diagnosis

Pictures and Scans of the Chest

Imaging plays an important role in checking for scarring and other signs linked to idiopathic pulmonary fibrosis (IPF) and other forms of lung damage. Doctors start with a chest X-ray, which creates a simple image of the lungs.

Test Name	What It Does	Signs It May Show
Chest X-ray	Creates a basic lung image with X-rays	Scarring, honeycombing
CT scan	Gives detailed cross-sectional images	Lung tissue patterns, damage
Echocardiogram	Uses sound waves to make heart pictures and videos	High blood pressure in lungs

This sometimes shows patterns of scarring or signs such as honeycombing, which are linked to pulmonary fibrosis. However, not all people with fibrosis show changes on a chest X-ray, especially in the early stages.

A high-resolution computed tomography (CT) scan uses many X-ray images taken from several angles to make detailed pictures of the lungs. This test gives a clearer view than a normal X-ray.

It helps doctors spot typical signs of IPF by showing the scarring and changes in tissues that are hard to see with other tests.

High-resolution CT also shows different damage patterns, making it easier to tell pulmonary fibrosis apart from other causes of shortness of breath or chronic cough.

An echocardiogram uses sound waves to look at the heart’s structure and function. It helps doctors find out if high pressure is present in the arteries of the lungs, which can happen with lung scarring.

This test does not find the scarring itself, but it shows how lung changes may affect the heart.

Measuring How the Lungs Work

Lung function tests, or pulmonary function tests, help doctors see how well the lungs are working. Several tests are often used, such as:

Spirometry: A person takes a deep breath and blows out hard into a tube. The test measures how much air can be breathed out and how fast it moves. People with scarring often have lower lung volumes and slow airflow.
Lung Volume Test: This test checks the total amount of air in the lungs at different points in breathing. Scarring usually makes the lungs hold less air than normal.
Diffusion Test: Air moves from the lungs into the bloodstream. This test measures how well this process is happening by checking how quickly gases like oxygen move through the lungs.
Pulse Oximetry: A small device clips to the finger and checks how much oxygen is in the blood.
Exercise Stress Test: Doctors might want to see how the lungs and heart work when you are active, such as walking on a treadmill or riding a stationary bike.
Arterial Blood Gas Test: A sample of blood from an artery is checked for oxygen and carbon dioxide levels. This test is usually done on the wrist.

Doctors look at numbers from these tests to see if symptoms like shortness of breath, dry cough, or fatigue are likely caused by scarring in the lungs.

Main Symptoms Checked During Lung Testing

Difficulty breathing or breathlessness
Chronic or dry cough that doesn’t go away
Fatigue, especially during activity

Taking a Sample from the Lungs

Sometimes, lung function tests and scans do not provide a clear diagnosis. In these cases, doctors may need a tissue sample, or biopsy, to find out what is happening in the lungs.

There are a few ways to collect lung tissue:

Surgical Tissue Sampling: A surgeon removes a small piece of lung through one or more cuts between the ribs. This can be done using a small camera and instruments, a method called video-assisted thoracoscopic surgery (VATS). VATS uses smaller cuts and usually leads to quicker healing. Sometimes, an open surgery called a thoracotomy is done, which requires a bigger cut.
Bronchoscopy: A doctor passes a thin, bendable tube called a bronchoscope into the lungs through the mouth or nose. Small tools at the end of the bronchoscope can take tiny tissue samples (transbronchial biopsy). The samples are small, but this method has fewer risks than surgery and can help rule out infections or other disorders.
Bronchoalveolar Lavage: During bronchoscopy, a doctor might also flush a part of the lung with liquid, then remove it for study. This helps look at cells and materials from deeper inside the lungs.

Surgical and non-surgical biopsies show specific features, such as thickening of tissues or longstanding scarring, helping doctors confirm or rule out idiopathic pulmonary fibrosis.

Tissue Sampling Methods at a Glance

Method	How It’s Done	What It Finds
VATS	Minimally invasive surgery with a camera	Clear tissue samples
Thoracotomy	Open chest surgery	Larger samples
Bronchoscopy	Tube passed into lungs to collect small samples	Fewer risks, smaller samples
Bronchoalveolar lavage	Washing lung with fluid to analyze collected material	Finds cells, infections

Blood Analysis

Doctors use blood tests to look for other possible causes of symptoms such as breathlessness, cough, or fatigue.

They might check how well the liver and kidneys are working, since problems with these organs can cause some of the same symptoms as pulmonary fibrosis.

Blood analysis can also point to autoimmune diseases or infections that could be behind the scarring, rather than idiopathic (unknown cause) pulmonary fibrosis. While blood tests alone cannot diagnose IPF, they help rule out other medical conditions.

Some blood tests doctors may order include:

Kidney function tests
Liver function tests
Tests for signs of inflammation or autoimmune disease

Normal results on these tests may prompt doctors to look more closely at the lungs as the main source of problems. These tests make sure the diagnosis fits the symptoms and test results.

Blood Test Checklist

Checks for other diseases that mimic pulmonary fibrosis.
Monitors how other organs are doing.
Helps guide other diagnostic steps.

Doctors bring together information from these scans, breathing tests, biopsies, and blood work to find out exactly what is causing symptoms like shortness of breath and cough.

The results also help plan which treatments to try and allow doctors to track how the condition changes over time.

Treatment

Prescription Treatments

Doctors may prescribe antifibrotic medications for some people with pulmonary fibrosis. These medications are approved for idiopathic pulmonary fibrosis and, in some cases, for other progressive forms of the disease.

Their main goal is to slow the worsening of lung function over time.

Side effects are common with antifibrotic therapy:

One type of antifibrotic may cause diarrhea or nausea.
Another antifibrotic may lead to nausea, reduced appetite, or skin sensitivity to sunlight that can cause a rash.

Patients using antifibrotic therapy require regular liver function monitoring to check for potential complications.

Other supportive treatments, such as acid-reducing medications for managing reflux symptoms, may also be recommended. While new therapies are under investigation, many are still being studied and are not yet approved for routine use.

Using Supplemental Oxygen

Oxygen support does not repair lung scarring, but it brings several benefits. It helps make breathing and physical activity a bit easier. Supplemental oxygen can also help prevent complications that happen when blood oxygen is too low.

Benefits include:

Less strain on the heart’s right side
Better sleep quality
Feeling less tired

People might use oxygen only when exercising or sleeping, but some need it all day. Portable tanks or concentrators help patients stay mobile and active.

Exercise and Breathing Programs

Pulmonary rehabilitation is a special program designed to support people with lung fibrosis. This approach is recommended because it provides practical help to improve day-to-day life.

These programs include:

Main Areas	What’s Included
Exercise Training	Builds physical strength, stamina
Breathing Techniques	Teaches ways to use oxygen better
Diet Support	Guidance about nutrition, meal planning
Emotional Help	Counseling and mental health support
Education	Information about pulmonary fibrosis

Attending pulmonary rehab may help people breathe better, feel fitter, and manage feelings linked to chronic illness.

Managing Sudden Worsening

Sometimes symptoms of pulmonary fibrosis become suddenly much worse, which is called an acute flare-up. In these moments, patients may urgently need more oxygen. They might also need to stay in the hospital for extra care.

Doctors could provide corticosteroids, antibiotics, or other medications to treat infection or reduce inflammation. If breathing becomes too difficult, a machine may be required to help the person breathe (mechanical ventilation).

Lung Transplant as a Choice

For some, a lung transplant becomes an option. Transplantation can lead to a better quality of life and in some cases, longer survival. However, the operation and recovery have risks, such as organ rejection and infections.

After a transplant, lifelong medication is needed to stop the body from attacking the new lung(s). People with advanced pulmonary fibrosis may discuss this choice with their healthcare team to see if a transplant could help their situation.

Lifestyle Changes and Practical Tips

Managing pulmonary fibrosis involves making everyday choices to support lung health and general well-being. The following suggestions can help people live better with this condition:

Quit Smoking: Stopping smoking is crucial. Avoiding tobacco smoke and secondhand smoke helps protect the lungs.
Reduce Exposure to Lung Irritants: Try to steer clear of indoor and outdoor substances that can affect breathing. This includes strong fumes from cleaning products, fuel, dust, and car exhaust. Making your home a smoke-free and well-ventilated space can lower the risk of irritation.
Eat for Energy and Strength: Eating can be difficult for some because breathing takes a lot of effort. It may help to eat smaller, more frequent meals rich in nutrients and calories. Consulting with a dietitian can provide more ideas for balanced meals and snacks.

Meal Planning Tips	Examples
Small, frequent meals	5-6 smaller meals a day
Nutrient-rich foods	Fruits, vegetables, lean meats
Quick snacks	Yogurt, cheese, nuts

Stay Physically Active: Exercise is important for lung health. Activities such as walking or cycling can improve stamina and reduce stress. It’s smart to talk with a healthcare provider before starting new activities. For those who need to limit walking, chair exercises or gentle movements, like tai chi, are good alternatives.
Prioritize Rest and Quality Sleep: Regular rest helps conserve energy. Fatigue is common with lung problems, so it’s helpful to take breaks and create a restful sleep routine. If sleep issues occur, seeking advice from a medical provider is a good step.
Stay Up-to-Date on Vaccinations: Illnesses such as the flu, pneumonia, and COVID-19 can make symptoms worse. It’s important to stay vaccinated against these infections. Encouraging family members to get vaccinated offers added protection. Avoiding crowded places during outbreaks can further decrease the risk of infection.
Follow the Care Plan: Sticking to prescribed treatments, attending appointments, and taking medicines as directed are all key. Pulmonary rehabilitation and regular check-ins help track changes and adjust care as needed.

Staying connected with a healthcare team and following these habits can support daily life and make it easier to cope with the challenges of pulmonary fibrosis.

Coping and support

People living with pulmonary fibrosis often face emotional and social challenges. Stress, worry, and sadness are common feelings. It is helpful for patients to talk openly about their situation and needs with loved ones.

Joining a support group can make a difference. These groups let people share experiences, ask questions, and learn tips for daily life. Talking with others who have pulmonary fibrosis can help reduce feelings of isolation.

Tips for support and care:

Stay connected with friends and family for encouragement and understanding.
Discuss symptoms like weight loss or clubbing with healthcare providers regularly.
Consider talking to a counselor or mental health expert for added emotional support.
Ask about palliative care to manage pain or discomfort as the disease progresses.

Ways to Cope	Helpful Actions
Emotional Well-being	Talk with others and join a support community.
Physical symptoms	Report changes like weight loss promptly.
Support system	Involve family, friends, and health professionals.

Getting Ready for Your Visit

Steps You Can Take Before Your Appointment

You can come prepared when meeting with a lung specialist. Bring a friend or family member to help with taking notes and provide emotional support.

Make a list before the meeting to share clear information and ask important questions.

Checklist for Preparation:

Write Down Symptoms: Note all symptoms such as cough, chest pain, shortness of breath, or fatigue. Record when these started and how they have changed.
Medical History: List any past or current health problems, hospital visits, and any diagnoses (such as connective tissue diseases).
Work and Lifestyle Details: Record past jobs, especially if you worked with dust or chemicals, as some jobs raise your risk of lung disease.
All Medicines and Supplements: List everything you take, including over-the-counter medicines, vitamins, and herbs, with doses.
Family History: Note if any relatives have lung problems, since family history can be a risk factor.
Questions for the Doctor: Write down what you want to know, such as possible causes, which tests the doctor will perform, risks linked to pulmonary fibrosis, treatment options, or how other health issues may change.

Item	Details to Prepare
Symptoms	Type, start date, changes
Medical history	Past issues, hospital stays
Work exposures	Past and current job roles
Medications & supplements	Names and doses
Family lung disease	Any relatives with lung conditions
Personal questions	Written down for the appointment

Questions Your Healthcare Provider May Ask

During the visit, the healthcare provider will likely ask for details about symptoms and health history.

They may focus on factors that can raise the risk for pulmonary fibrosis, such as exposure to certain jobs, previous chest radiation, smoking, or past chemotherapy.

Common topics include:

The nature, timing, and severity of symptoms like coughing, chest pain, or breathlessness.
Treatments for other illnesses, especially connective tissue diseases.
Past and current medicines or supplements.
Tobacco use history and duration.
Exposure to harmful substances at work or home.
Family members with long-term lung disease.
Any history of chemotherapy or chest radiation.

Patients should answer these questions as clearly as possible to help the team make a careful diagnosis and plan next steps.

Pulmonary Fibrosis – Diagnosis and Treatment