Pulmonary fibrosis - Symptoms and causes

Pulmonary fibrosis is a serious lung condition where the tissue deep inside the lungs becomes damaged and scarred. The scarring then causes the lungs to stiffen, which impairs their ability to expand properly and take in sufficient oxygen. 

Over time, the scarring worsens, and people often find it increasingly difficult to breathe. It is not an infection or cancer, and it cannot spread from one person to another. The condition can progress slowly or quickly, depending on its cause & the individual's overall health.

Some people develop pulmonary fibrosis due to prolonged exposure to environmental irritants, like the dust or smoke. In contrast, others may get it as a side effect of certain medications, autoimmune conditions, or radiation therapy. In many cases, however, no clear cause is found. These cases are known as idiopathic pulmonary fibrosis. 

Symptoms

• Shortness of breath, especially during activity
• Persistent dry cough
• Fatigue
• Unexplained weight loss
• Aching muscles and joints
• Clubbing (widening and rounding) of fingertips or toes

Common Causes 

• Long-term exposure to harmful particles

Regular inhalation of substances like silica dust, asbestos fibers, coal dust, or metal particles can lead to lung irritation and scarring over time

• Certain medications

Some drugs used in chemotherapy (such as bleomycin), heart medications (like amiodarone), and certain antibiotics (like nitrofurantoin) are known to cause lung damage in rare cases

• Radiation therapy to the chest

People who undergo radiation for cancers like breast or lung cancer may develop fibrosis months or years after treatment, especially if high doses were used

• Autoimmune diseases

Conditions in which the immune system attacks the body, such as rheumatoid arthritis, systemic sclerosis (also known as scleroderma), and polymyositis, can lead to chronic inflammation in the lungs and ultimately result in scarring

• Viral infections

Some viral illnesses have been linked to the development of pulmonary fibrosis, though this is less common

• Genetics and family history

A small number of people inherit genetic mutations that increase their risk of developing fibrosis, particularly if more than one family member has been affected

• Idiopathic pulmonary fibrosis (IPF)

In many cases, no cause is found despite thorough testing. These are labeled idiopathic, and IPF is the most common and studied form of this type

Who's More Likely to Be Affected

• Age above 60
• Male gender
• Smoking (current or past)
• Long-term exposure to dust, fumes, or pollutants
• Working in mining, farming, construction, or metalworking
• History of viral infections
• Use of certain medications over time
• Undergoing chest radiation therapy
• Presence of autoimmune diseases
• Family history of pulmonary fibrosis
• Genetic mutations linked to lung disease

Complications of Pulmonary Fibrosis 

• Worsening breathlessness

As scarring progresses, lungs lose their ability to take in oxygen, making even mild activity exhausting over time

• Low blood oxygen levels (chronic hypoxemia)

Long-term oxygen deprivation can lead to fatigue, confusion, and damage to vital organs

• Pulmonary hypertension

High pressure in the arteries of the lungs forces the heart to work harder, which can lead to heart strain or failure

• Right-sided heart failure (cor pulmonale)

The right side of the heart may weaken due to persistent high pressure in the lungs, causing swelling in the legs, liver, and abdomen

• Frequent lung infections

Reduced lung function raises the risk of respiratory infections, which can be severe or recurrent in nature

• Lung collapse (pneumothorax)

Rare but serious, it occurs when air leaks in the space between the lung & the chest wall

• Blood clots

Limited mobility and poor lung function can increase the chances of developing blood clots, particularly in the legs or lungs

• Lung cancer

People with pulmonary fibrosis, particularly idiopathic forms, may have a higher chance of developing lung cancer over time

How Is Pulmonary Fibrosis Diagnosed

• Medical history and symptom check

The doctor asks about breathing problems, cough, work history, past illnesses, and any family members with lung disease

• Physical examination

Using a stethoscope, the doctor listens for “crackles” in the lungs, often described as Velcro-like sounds during breathing

• Pulmonary function tests (PFTs)

These measures how well the lungs hold air and how efficiently oxygen moves into the blood. A reduced lung capacity may suggest fibrosis

• Oxygen level check

A pulse oximeter or arterial blood gas test is used to determine the amount of oxygen circulating in the bloodstream

• High-resolution CT (HRCT) scan

This imaging test provides detailed views of lung tissue. It can reveal scarring patterns specific to pulmonary fibrosis, especially in idiopathic cases

• Chest X-ray

It may show lung damage or rule out other lung problems, but it’s not detailed enough to confirm fibrosis on its own

• Exercise testing (e.g., 6-minute walk test)

Measures how well the lungs function during physical activity and whether oxygen levels drop with exertion

• Blood tests

Help rule out autoimmune diseases or infections that may be causing lung symptoms

• Bronchoscopy with lavage

A tube is inserted into the lungs to collect some fluid. This helps rule out infections or detect inflammatory cells

• Lung biopsy (surgical or through bronchoscopy)

In some cases, a small tissue sample is needed to examine the type and extent of lung scarring under a microscope, especially when imaging is unclear

Management and Treatment

• Antifibrotic medications

Drugs like pirfenidone and nintedanib (for idiopathic pulmonary fibrosis) can slow the progression of scarring, though they do not reverse existing damage

• Oxygen therapy

Supplemental oxygen helps maintain adequate oxygen levels, reduces breathlessness, and supports daily activity, especially in advanced stages

• Pulmonary rehabilitation

A supervised program that includes breathing exercises, physical conditioning, and nutritional counseling to improve lung function and energy levels

• Cough management strategies

Since cough can be persistent, patients may benefit from speech therapy or medications to reduce the urge to cough

• Treatment of associated conditions

Conditions like gastroesophageal reflux disease (GERD) or pulmonary hypertension are often managed alongside fibrosis to minimize complications

• Vaccinations

Staying up to date with flu, pneumonia, and COVID-19 vaccines helps prevent infections that could further harm lung function

• Lung transplant

In select patients with severe disease and good overall health, lung transplantation is considered when other treatments no longer help

• Psychological support and palliative care

Counseling, support groups, and palliative care services can help manage emotional distress, especially as the disease progresses

Day-to-Day Self-Care

• Follow your treatment plan strictly.

Take medications on time, attend all follow-up appointments, and report any new or worsening symptoms immediately

• Use oxygen as directed

If supplemental oxygen is prescribed, use it consistently—even if symptoms seem mild—to avoid low oxygen levels that can silently worsen health

• Avoid exposure to lung irritants

Avoid exposure to smoke, dust, strong fumes, and areas with poor air quality. Wear a protective mask if exposure is unavoidable

• Stay physically active within limits

Gentle exercises, as recommended by a doctor or rehabilitation specialist, can help maintain stamina and alleviate breathlessness

• Eat balanced, small meals

Nutritious meals support energy levels and overall health. Smaller, more frequent meals may also help minimize breathlessness during eating

• Manage stress and emotional health

Practice relaxation techniques, such as deep breathing, mindfulness or meditation, to promote relaxation. Speak to a counselor or join a support group if needed

• Prevent infections proactively

Wash your hands regularly, avoid close contact with individuals who are sick, and stay up to date with all recommended vaccines

• Rest when needed

Fatigue is common. Plan your day with rest breaks and prioritize essential activities to conserve energy

• Monitor your oxygen levels

If advised, use a pulse oximeter at home to monitor oxygen saturation and detect early signs of low oxygen levels

Sterling Hospital is Your Partner in Better Health Across Gujarat

At Sterling Hospital, we provide the utmost care and comfort throughout your journey to a healthier life. We've got you covered if you are looking for the best pulmonologist in Vadodara, Rajkot, or  Gandhidham. 

Our pulmonary department is built with a team of the best pulmonologists in these regions to provide premium treatment and the best results. Contact Sterling Hospital to schedule a consultation and embark on a healthier future.