What is Lung Fibrosis (Pulmonary Fibrosis)? What Are Its Symptoms? How Is It Diagnosed and Treated?

Lung fibrosis, which is diagnosed in approximately 50,000 people worldwide each year, is a rare disease that causes thickening and hardening of the lung tissue. This condition causes the lungs to lose their elasticity and makes the passage of oxygen into the bloodstream difficult. Symptoms are mild at first and progress over time.

In this article, you will find everything you need to know about this disease, including what lung fibrosis is, the symptoms of lung fibrosis, the causes of lung fibrosis, and lung fibrosis treatment options.

What is lung fibrosis?

Lung fibrosis is a chronic condition characterized by the formation of scar tissue and thickening in the lungs. Normally possessing a spongy and flexible structure, the lung tissue hardens and loses its elasticity in this disease. This process, referred to as scarring, makes it difficult for the lungs to perform their basic functions.

Changes in lung tissue

The air sacs (alveoli) in your lungs normally have thin, lace-like walls. In lung fibrosis, these walls thicken and become covered with scar-like tissues. While healthy lung tissue is soft and flexible enough to allow you to breathe easily, damaged tissue becomes rigid.

Previously, it was thought that this condition stemmed from an inflammatory response in the lungs. However, it is now estimated that the damage occurring in the lungs enters an abnormal healing process and becomes scarred. The exceeding of a certain level of scarring is defined as pulmonary fibrosis.

This scar tissue formation prevents the lungs from filling with air. During breathing, atmospheric oxygen cannot be taken up by the damaged tissue and cannot pass into the bloodstream. Oxygen does not adequately reach the vessels around the alveoli, and this condition affects all the systems of your body.

Course and effects of the disease

Lung fibrosis is a progressive disease. The changes occurring in the damaged tissue develop slowly over months or years. The amount of scar tissue increases over time, and this process is generally irreversible. Loss of function exhibits a progressive characteristic in most patients.

The disease may progress without causing any complaints in the early stages. However, as the lung tissue thickens and shrinks, your body cannot get enough oxygen. This condition leads to serious problems ranging primarily from respiratory failure to heart failure.

Oxygen is a necessary molecule for all the functions of your body. When lung fibrosis develops, you cannot get enough oxygen, and the elimination of carbon dioxide is also impaired. Consequently, failures in various organ systems may arise.

The rate of progression of the disease varies from person to person. While symptoms start rapidly in some patients, they progress more slowly in others. It is difficult to precisely predict the course of the disease. Factors such as your age and accompanying health problems affect the progression of the disease.

What are the symptoms of lung fibrosis?

The symptoms of lung fibrosis appear insidiously at first, and most patients do not notice their complaints in the early period. Symptoms develop slowly and tend to worsen over time. The rate of progression of the disease and the severity of symptoms may vary from person to person.

Early symptoms

When the disease picture first forms, the most common symptom is shortness of breath. Initially, this condition manifests itself only during physical activity. You experience breathlessness when climbing stairs or walking briskly.

A persistent dry cough is another symptom of the early period. This cough has a persistent and recurrent characteristic. Continuing without producing phlegm, this cough can be bothersome.

Feelings of fatigue and weakness affect your daily activities. You feel exhausted in tasks you previously performed easily. In elderly individuals, these symptoms can be attributed to age or immobility. Therefore, the disease diagnosis may be missed, and a delay in diagnosis occurs.

When listening to your lungs, your doctor may hear crackle-like wheezes resembling the sound of Velcro. This sound is a sign of changes in the lung tissue.

Advanced stage symptoms

As the disease progresses, symptoms become more pronounced. Shortness of breath occurs even during daily activities. You become breathless during a short walk or while doing simple household chores.

Widening and rounding are observed at the tips of your fingers or toes. This condition, referred to as clubbing, manifests itself as the enlargement of the fingertips and the curving of the nails toward the fingertips. You or your doctor can easily notice this change.

You experience unintended weight loss due to loss of appetite. Your body cannot get adequate nutrition and loses strength. Muscle and joint pain are also among the symptoms that appear in the advanced stage.

You may feel a sense of discomfort or pain in your chest. Dryness of the mouth and eyes is observed in some patients. Circulatory disorders in the fingers and toes may also accompany this.

Effects of symptoms on daily life

Shortness of breath severely affects your daily activities. This condition, which develops with exertion, reduces your quality of life. Complaints that are mild at first reach a disruptive dimension as the disease progresses.

Your shortness of breath increases during physical activities. You struggle even while performing simple tasks. This condition reduces your independence and alters your daily routine.

Chronic cough prevents you from resting and negatively affects your social life. The constant feeling of fatigue strains your work performance and family relationships. In the later periods of the disease, you may need assistance.

Since symptoms can be confused with other diseases, early diagnosis is important. If you are experiencing a dry cough, shortness of breath upon exertion, and fatigue, you should consult a pulmonologist without wasting time.

Causes of lung fibrosis

Many different factors can lead to the development of lung fibrosis. However, in a significant portion of cases, the exact cause cannot be determined. Environmental exposure, genetic predisposition, and certain health problems play a role in the formation of the disease.

Idiopathic pulmonary fibrosis

Among the causes of lung fibrosis, the most frequently encountered condition is idiopathic pulmonary fibrosis. The word idiopathic means that the factor causing the disease is not precisely known. In about half of the patients diagnosed, the underlying cause cannot be detected.

This disease is mostly seen in individuals over the age of 50 and is more frequent in men. It is expected to develop in 1 out of every 1000 people. 90% of IPF patients have a history of smoking. Factors such as viral infections and reflux have been thought to be effective in the development of the disease, but they could not be definitively proven.

Environmental factors and occupational exposures

Long-term exposure to certain substances can harm your lungs. The risk of developing the disease is high in those exposed to metal dust (lead, steel, brass) and wood dust. Individuals working in professions such as mining, farming, stone polishing, and hairdressing are at risk.

Silica dust, asbestos fibers, coal dust, and grain dust cause damage to the lung tissue. Bird and animal droppings are also among harmful substances. The finding of increased numbers of inorganic particles in the lymph nodes of patients with pulmonary fibrosis in autopsies is an important finding supporting environmental exposure.

Autoimmune diseases and infections

In autoimmune diseases such as rheumatoid arthritis, scleroderma, and lupus, your body may attack its own lung tissue. Dermatomyositis, polymyositis, and sarcoidosis also lay the groundwork for the development of lung fibrosis. In these diseases, your immune system perceives the lung tissue as foreign.

Certain microbiological agents play a role in the onset of the disease. The frequency of herpes virus in the IPF lung has been reported as 30-100%. Both protein and DNA of the Epstein-Barr virus have been detected in the alveolar epithelial cells of patients. Viral infections such as Hepatitis C and adenovirus can also contribute to the development of fibrosis.

Genetic predisposition and familial inheritance

The risk of contracting the disease is higher in individuals with a family history of lung fibrosis. Genetic predisposition has been detected in 35% of IIP patients. In familial pulmonary fibrosis cases, the disease is seen in more than one individual in the same family.

MUC5B, TOLLIP, TERT, and TERC genes are associated with the disease. These genes affect the repair processes of the lung tissue. Familial cases are thought to have low penetrance autosomal dominant inheritance. Familial forms tend to appear at a younger age.

Drug use and radiation

Some chemotherapy drugs can damage lung tissue. Drugs used in cancer treatment, such as methotrexate and cyclophosphamide, can lead to fibrosis. Heart rhythm-regulating amiodarone, the antibiotic nitrofurantoin, and certain anti-inflammatory drugs also pose a risk.

Some individuals receiving radiation therapy for lung or breast cancer show signs of lung damage months or years after treatment. The severity of the damage depends on how much of the lung was exposed to radiation, the total amount of radiation applied, and whether chemotherapy was used.

How is lung fibrosis diagnosed?

Various tests and evaluations are performed to make an accurate diagnosis. The diagnostic process is generally carried out by an expert chest diseases physician and involves multiple methods.

Physical examination and medical history evaluation

Your doctor first listens to your complaints and inquires about your medical history. They ask questions such as whether there is a history of lung disease in the family, your smoking habits, and whether you have been exposed to chemical substances in the workplace. They obtain information about workplace working conditions and living conditions.

During the physical examination, your lungs are listened to with a stethoscope. When asked to take a deep breath, crackle-like or Velcro-like wheezes may be heard in the lung sounds. These sounds are an important sign of changes in the lung tissue. In addition, it is checked whether there is clubbing in your fingers.

Imaging methods

Chest X-ray is the initial evaluation tool. It is used to detect abnormalities in the lungs. However, while this method is useful in monitoring the course of the disease, it is mostly insufficient for diagnosis alone.

High-resolution computed tomography (HRCT) plays a key role in diagnosis. This scan shows detailed changes in the lung tissue and helps determine the exact location and size of the scar tissue. It provides great benefit in determining the degree of lung damage.

In some cases, echocardiography is requested. Cardiac ultrasonography may be performed to detect and monitor conditions such as pressure elevation in the right side of the heart and heart failure that appear in the advanced periods of the disease.

Respiratory function tests

These tests are used to evaluate how well your lungs are working. Lung capacity and airflow are measured with spirometry. Test results can be compared over time to monitor the progression of the disease.

Measurements of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) are performed. DLCO shows how well the lungs transfer oxygen into the blood. In the early stage of the disease, the parameter that deteriorates earliest is DLCO. Blood oxygen level measurement and the 6-minute walk test are also among the tests applied.

Blood tests and laboratory examinations

Blood tests are used to measure inflammatory markers and oxygen levels. They are performed to rule out other possible causes such as infections and autoimmune diseases.

The presence of specific antibodies helps determine whether the disease has an autoimmune origin. Connective tissue antibodies such as rheumatoid factor and antinuclear antibody (ANA) are checked routinely.

Lung biopsy

When necessary, a sample is taken from the lung tissue and examined under a microscope. This is an important step to make a definitive diagnosis. In some patients, a diagnosis can be made via lung tomography, and a biopsy may not be deemed necessary.

Biopsy is generally performed through a flexible camera following a small incision made in the chest wall under general anesthesia. The outer surface of the lung is visualized, and a tissue sample is taken from the appropriate area. In 34% of the disease, the diagnosis is confirmed by surgical lung biopsy.

The obtained biopsy is subjected to histopathological examination. A sample can also be taken from the lung tissue via the bronchoscopy method. This procedure is critical for ruling out other diseases and making an accurate diagnosis.

Lung fibrosis treatment

Lung fibrosis treatment cannot reverse the damage that has occurred. However, new medications can slow down the progression of the disease. The aim of treatment is to reduce complaints and increase your quality of life.

Medication treatment options

Antifibrotic drugs slow down the scarring process in the lung tissue. Pirfenidone and nintedanib aim to slow the progression of the disease. These drugs help reduce the formation of scar tissue.

Corticosteroids are used to reduce inflammation. However, their long-term use can cause side effects. Immunosuppressive drugs have a place in the treatment of pulmonary fibrosis associated with autoimmune diseases. These drugs try to prevent your body from attacking the lung tissue.

Corticosteroids and immunosuppressive drugs provide limited benefit. Your doctor determines the most appropriate drug combination according to your condition.

Oxygen therapy

Oxygen therapy is applied to increase the oxygen level in the blood and alleviate shortness of breath. Especially in advanced-stage patients, quality of life can be increased by providing oxygen support.

Portable oxygen devices help you maintain your daily activities. With these devices, you can go outside and continue your social life. Oxygen therapy is important to increase oxygen levels in the lungs.

Pulmonary rehabilitation programs

Pulmonary rehabilitation is performed to improve your respiratory functions and elevate your quality of life. These programs provide exercise, education, and support. They include breathing techniques, physical exercises, and nutritional counseling.

Daily exercise training, breathing exercises, and nutritional education should be applied. Rehabilitation programs increase the respiratory capacity of patients and develop their exercise endurance.

Pulmonary rehabilitation programs should become accessible in every province. They should include not only physical exercise but also components of nutritional counseling, psychological support, and education. The program should be applied for at least 8 weeks, two or three sessions per week.

Lung transplantation

Lung transplantation can be considered in advanced-stage patients and individuals meeting appropriate criteria. It involves replacing the diseased lung with a healthy donor lung. It is an important treatment option that extends lifespan and increases quality of life.

Lung transplantation can be the most effective form of treatment in suitable patients. However, it is a major surgical procedure and requires lifelong use of immunosuppressive drugs to prevent organ rejection. Therefore, the decision must be made by performing a careful evaluation.

Lifestyle changes and supportive care

If you smoke, you must quit. Smoking can accelerate the progression of the disease. It is important for you to avoid environmental factors that may cause discomfort.

You need to eat healthy and exercise regularly. Routine vaccinations such as the pneumonia vaccine and the annual flu vaccine are recommended. You should take precautions to protect against respiratory tract infections.

The treatment process requires a multidisciplinary approach. Since every patient's condition is different, making individualized care plans is of great importance. Regular control intervals, lung function tests, and exercise recommendations should be prepared specifically for the patient.

Frequently asked questions about lung fibrosis

Questions of interest regarding the disease are generally similar. To clear up question marks in the minds of patients and their relatives, we answered the most frequently asked questions.

Is lung fibrosis fatal?

Many diseases that are not diagnosed early and closely monitored can be fatal. Pulmonary fibrosis is also one of these diseases. The prognosis of the disease is worse than many cancers.

However, this condition does not progress the same way in every patient. With treatment and monitoring, many patients can live longer. Idiopathic pulmonary fibrosis is a progressive and potentially life-threatening disease. Nevertheless, lifespan can be extended with early diagnosis and appropriate treatment.

Is lung fibrosis contagious?

No, lung fibrosis is not a contagious disease. The disease does not pass from one person to another. Being in the same environment or touching the patient does not pose any risk.

The disease generally develops through autoimmune processes, environmental exposures, or mechanisms of unknown cause. Therefore, there is no harm in establishing close contact with sick individuals.

How long do people with lung fibrosis live?

Lifespan varies from person to person and depends on many factors. Age, disease stage, and the applied treatment are the primary factors affecting duration. Some information on the internet mentions a 3-5 year lifespan after diagnosis.

Particularly, median survival is on average three years after diagnosis, and the five-year survival rate is reported as 20-30%. However, these figures are based on the past when treatment options were limited. Before new drugs were developed and pulmonary rehabilitation and lung transplantation became widespread, the lifespans of patients were not very long.

Today, thanks to the spread of lung transplantation and the application of other rehabilitation and drug treatments, patients can lead longer and more comfortable lives. The average lifespan after diagnosis is currently unknown.

Does smoking cause lung fibrosis?

Many people living with IPF have smoked in the past, but it is not known whether smoking is a direct cause of IPF. However, many studies have shown that smoking is a potential risk factor in the development of the disease.

Approximately 41-83% of IPF patients consist of individuals who smoke or have quit smoking. About 90% of cases are smokers. These data show that there is a strong relationship between smoking and the disease.

Does lung fibrosis recover completely?

There is currently no known cure for the disease. However, treatments are available to slow the progression of the disease and help manage symptoms effectively. Medications and other treatment methods cannot completely reverse the damage in the lungs.

Currently, lung transplantation, which is the only treatment option capable of providing a cure in IPF, is found suitable for only a very small number of patients. The main goal of treatment is to keep the disease under control and ensure that the quality of life does not deteriorate.

In summary, lung fibrosis

Although lung fibrosis is a progressive disease, you can preserve your quality of life with early diagnosis and the right treatment approach. When you notice symptoms such as shortness of breath and dry cough, you should consult a pulmonologist without wasting time. Although the disease does not heal completely, new medications, pulmonary rehabilitation, and lifestyle changes keep symptoms under control. Quitting smoking, going to regular check-ups, and adhering to the treatment plan recommended by your doctor are of great importance. Living with the disease can be difficult, but with the right approach, you can lead a more comfortable life.