Idiopathic Pulmonary Fibrosis (Lung Hardening): What It Is, Symptoms, and Treatment

Idiopathic pulmonary fibrosis (IPF) is a disease characterized by thickening and hardening of the lungs with an unknown cause. The term "unknown cause" refers to the inability to determine the cause of lung damage despite all examination and diagnostic methods. It is a condition where the lungs are damaged, leading to difficulty in breathing as the disease progresses. Idiopathic pulmonary fibrosis primarily affects older individuals, while it is rare in middle-aged and younger people. Although there is no complete cure for this disease, treatment methods can help manage the disease's progression and maintain a stable quality of life.

What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a lung disease that occurs when lung tissue is damaged or injured. The thickened, hardened tissue can hinder the lungs' functioning and may lead to conditions that cause shortness of breath as the situation progresses. Lung damage does not occur suddenly; it is a condition that progresses slowly over months or years. As a result of IPF, damage to lung tissue thickens over time, deviating from its normal structure and leading to a reduction in lung volume. Consequently, this results in insufficient oxygen intake and the inability to expel carbon dioxide from the body. Idiopathic pulmonary fibrosis does not have the same effects on everyone. In some patients, symptoms may be minimal, while in others, symptoms may be more severe and disrupt daily activities. In patients with idiopathic pulmonary fibrosis, acute exacerbations, also known as attacks, may occur from time to time. During acute exacerbations, treatments involving mechanical ventilation (devices that provide oxygen support to help the lungs perform vital functions) may be applied to patients. Additionally, doctors may prescribe short-term antibiotic and corticosteroid treatments during these exacerbation periods.

What Are the Symptoms of Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a slowly progressive disease that tends to worsen over time. The most prominent symptom is shortness of breath, which may not initially be concerning for many people. However, as the disease progresses, shortness of breath can significantly disrupt daily life. It is essential to consult a specialist if shortness of breath persists for more than three weeks. The primary symptoms of idiopathic pulmonary fibrosis are generally as follows:

• Shortness of breath
• Persistent dry cough
• Clubbing of fingers, described as round fingertips
• Loss of appetite and weight loss
• Fatigue and weakness
• Muscle and joint pain

In addition to these symptoms, idiopathic pulmonary fibrosis can also lead to other diseases or health issues. This can be referred to as complications of idiopathic pulmonary fibrosis. Some conditions that IPF may cause include:

• Respiratory Failure: This means a severe drop in oxygen levels in the blood and is one of the most advanced stages of IPF.
• Pulmonary Hypertension (high blood pressure in the lungs): This condition affects only the pulmonary arteries. Damage and hardening in the lungs compress the smallest arterial and capillary vessels, resulting in increased pressure in the pulmonary arteries and causing hypertension in the lungs.
• Heart Failure: This condition, which signifies right-sided heart failure, occurs when the heart has to pump more blood to move through obstructed pulmonary arteries.
• Lung Cancer: Untreated and rapidly progressing idiopathic pulmonary fibrosis may lead to the development of lung cancer.
• Other Lung Diseases: As pulmonary fibrosis progresses, it may lead to lung collapses, blood clots in the lungs, and lung infections.

Causes and Risk Factors of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis causes damage and thickening of the tissue between the alveoli, the air sacs in the lungs. As this condition progresses, it leads to inadequate oxygen transport in the blood and prevents the lungs from functioning properly. Idiopathic pulmonary fibrosis is a disease with an unknown cause that is rarely seen. However, some risk factors are associated with the disease. These primarily include prolonged exposure to toxic substances, particularly radiation therapies used in cancer treatment and certain medications used for specific diseases. Some general conditions that may lead to idiopathic pulmonary fibrosis include:

• Advanced age
• Genetic predisposition
• Smoking
• Male gender
• Adverse environmental factors (humidity, moisture, exhaust smoke, coal smoke, etc.)
• Being part of certain occupational groups (miners, hairdressers, animal caretakers, paint industry workers, etc.)
• Gastroesophageal reflux disease
• Radiation treatments
• Previous chemotherapy treatment
• Underlying lung diseases
• Use of certain medications (some chemotherapy drugs, heart medications, anti-inflammatory drugs, and antibiotics)
• Presence of certain diseases (such as pneumonia, mixed connective tissue diseases, scleroderma, rheumatic joint diseases, and polymyositis)

The causes mentioned above, along with many other unlisted factors, can contribute to the development of idiopathic pulmonary fibrosis. Additionally, viruses and microbial diseases can also be considered as potential causes. Even in the absence of smoking, being in an environment where smoking occurs can trigger IPF.

Diagnosis of Idiopathic Pulmonary Fibrosis

Establishing a definitive diagnosis of idiopathic pulmonary fibrosis can be challenging for doctors. This difficulty arises because IPF shares similar characteristics with chronic obstructive pulmonary disease (COPD) and various other lung diseases. Through examinations and evaluations, doctors approach patients for differential diagnosis using various methods. Patients presenting with specific complaints are first thoroughly interviewed regarding their symptoms, and a family history of lung disease is inquired about. Additionally, information about workplace conditions and living circumstances is gathered. Some of the methods used for diagnosing idiopathic pulmonary fibrosis include:

• Medical History and Physical Examination: The patient's smoking history, exposure to chemicals in the workplace, episodes of shortness of breath while walking, the presence of a crackling sound from the lungs, and any signs of clubbing fingers are assessed.
• Respiratory and Blood Tests: Respiratory function tests measure the amount of oxygen entering the lungs and how much oxygen the lungs hold. Blood tests determine how effectively the lungs transfer oxygen into the blood and the carbon dioxide levels in the blood.
• Chest X-ray and CT Scan: Chest imaging can reveal any potential cancer or fluid accumulation in the lungs. A CT (computed tomography) scan provides a more detailed view of the lungs and can identify the formation of scar tissue (fibrosis) in the lungs.
• Bronchoscopy: This method allows for detailed examination of lung tissue, bronchi, and the trachea by entering through the airways using an instrument called a bronchoscope. It also allows for the collection of tissue samples when necessary.
• Lung Biopsy: The biopsy procedure, the last resort for diagnosis, is performed under general anesthesia. During a lung biopsy, a thin, illuminated tube is used to obtain samples from lung tissue for examination.

Treatment of Idiopathic Pulmonary Fibrosis

There is no definitive and clear treatment for idiopathic pulmonary fibrosis. The main goal of treatment for this disease is to keep it under control, minimize the symptoms of the disease, and ensure the patient's quality of life does not deteriorate. To slow the progression of idiopathic pulmonary fibrosis and reduce its effects, some of the treatment methods and protective measures include:

• Quitting smoking
• Eating a healthy diet
• Engaging in regular exercise
• Consistently using medications prescribed by doctors, such as pirfenidone and nintedanib, to prevent the worsening of lung damage
• Using home oxygen masks
• Performing breathing exercises known as pulmonary rehabilitation to facilitate easier breathing
• Taking protective measures to avoid influenza or colds and working to maintain a strong immune system
• Getting vaccinated against the flu or pneumonia upon doctor's recommendation