Idiopathic Pulmonary Fibrosis (Lung Hardening): What is it, Symptoms, Treatment

Idiopathic pulmonary fibrosis (IPF) is literally a disease of lung thickening and hardening of unknown cause. The term “unknown cause” refers to the situation where the cause of the wound in the lung cannot be determined despite all kinds of examination and diagnostic methods. It is a disease in which the lungs are injured and breathing becomes difficult in later periods. Idiopathic pulmonary fibrosis is a rare disease in people of middle and young age, mostly affecting older people. Although there is no complete cure for this disease, treatment methods can be used to control the course of the disease and keep the quality of life stable.

What is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a lung disease that occurs when lung tissue is damaged or injured. The thickened hard tissue can make it difficult for the lungs to work, and as this condition progresses, it can cause discomfort, including shortness of breath. Damage to the lungs does not occur in a short time. This is a condition that progresses slowly over months or years. Damage to the lung tissue as a result of IPF thickens over time and moves away from its normal structure, causing the lungs to shrink. As a result of this situation, the problem of not getting enough oxygen and not being able to remove carbon dioxide from the body occurs. Idiopathic pulmonary fibrosis may not have the same effects on everyone. While in some patients the symptoms are almost non-existent, in some patients they may be more severe and disrupt daily activities. In idiopathic pulmonary fibrosis, these people may occasionally experience attacks called acute exacerbations. In acute exacerbations, treatments called mechanical ventilation (a device that provides oxygen support to the patient so that the lungs can perform their vital functions) can be applied to patients. Apart from this, doctors can also give patients short-term antibiotic and cortisone drug treatments during these attack periods.

What are the Symptoms of Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis is a disease that progresses slowly and tends to worsen over time. This disease, whose most prominent symptom is shortness of breath, may not be alarming for most people at first. However, as the disease progresses, shortness of breath may become disruptive to daily life. Especially in complaints of shortness of breath lasting longer than 3 weeks, a specialist doctor should be consulted. In general, the main symptoms of Idiopathic pulmonary fibrosis are as follows:

• Shortness of breath
• Persistent dry cough
• Round fingertips, described as clubbed fingers
• Loss of appetite and weight loss
• Fatigue, weakness
• Muscle and joint pains

Apart from these symptoms, idiopathic pulmonary fibrosis may also cause some diseases or health problems. This condition can also be called complications of idiopathic pulmonary fibrosis. Some diseases that IPF can cause are as follows:

• Respiratory Failure: This means that the oxygen levels in the blood are severely reduced. This condition is one of the most advanced levels of IPF.

• Pulmonary Hypertension (high blood pressure in the lungs): A condition that affects only the arteries in the lungs. Scars and hardening in the lungs compress the smallest arteries and capillaries. In this case, the pressure in the pulmonary arteries increases and creates hypertension in the lungs.

• Heart Failure: This condition, which means right-sided heart failure, means the heart has to pump more blood to move blocked lung arteries.

• Lung Cancer: Idiopathic pulmonary fibrosis, which is left untreated for a long time and progresses rapidly, may cause lung cancer.

• Other Lung Diseases: As Pulmonary Fibrosis progresses, lung collapse, blood clots in the lung and lung infections may occur.

Idiopathic Pulmonary Fibrosis Causes and Risk Factors

Idiopathic pulmonary fibrosis causes the tissues between and around the air sacs called alveoli in the lungs to become injured and thicken over time. As this condition progresses, it causes the oxygen ingested to not be transported to the blood at a sufficient rate and the lungs cannot perform their functions properly. Idiopathic pulmonary fibrosis is a rare disease whose cause is unknown. However, although the exact cause is not known, there are some risk factors that may cause the disease. These include exposure to toxic substances for a long time, radiation treatments used especially in cancer treatment, and drugs used in certain diseases. In general, some conditions that may cause Idiopathic pulmonary fibrosis are as follows:

• advanced age
• Genetic predisposition
• smoking
• male gender
• Negative environmental factors (humidity, humidity, exhaust smoke, coal smoke, etc.)
• Being a member of some professional groups (miner, hairdresser, animal care, paint industry, etc.)
• reflux
• radiation treatments
• Having previously received chemotherapy treatment
• Underlying lung diseases
• Use of certain medications (some chemotherapy drugs, heart medications, anti-inflammatory drugs and antibiotics)
• Presence of certain diseases (some muscle-joint diseases such as pneumonia, mixed connective tissue diseases, scleroderma, rheumatic joint disease and polymyositis)

These reasons and many other uncountable factors may cause idiopathic pulmonary fibrosis. Apart from these, viruses and microbial diseases can also be considered as causes. Even if there is no smoking, being in an environment where smoking can also trigger IPF.

Diagnosis of Idiopathic Pulmonary Fibrosis

Making a definitive diagnosis of idiopathic pulmonary fibrosis can be challenging for doctors. This is because IPF has similar features to chronic obstructive pulmonary disease (COPD) and various other lung diseases. With the examination and evaluations performed, doctors approach patients with some methods for differential diagnosis. For patients who apply with certain complaints, their complaints are first taken thoroughly and they are questioned about whether there is a lung disease in their family. Apart from this, information is also obtained about workplace working conditions and living conditions. Some of the methods used when diagnosing Idiopathic Pulmonary Fibrosis are:

• Medical History and Physical Examination: It is determined whether the patient smokes, has been exposed to chemicals at work, or is short of breath while walking, or whether there is a crackling sound coming from the lungs, or a pattern similar to clubbing of the fingers.

• Respiratory and Blood Tests: Respiratory function tests determine the amount of oxygen entering the lungs, how much oxygen the lungs hold, and blood tests determine how well the lungs transmit oxygen to the blood and the carbon dioxide rate in the blood.

• Chest X-ray and CT Scan: A chest scan can detect possible cancer or fluid accumulation in the lungs. CT (computed tomography) scan provides a more detailed view of the lungs and scarring (fibrosis) formation in the lung can be determined.

• Bronchoscopy: It is a method that allows detailed examination of the lung tissue, bronchi and trachea by entering the respiratory tract with an instrument called a bronscope, and, when necessary, taking samples from the lung tissue.

• Lung Biopsy: The biopsy procedure, which is the last resort method, is performed under general anesthesia. With lung biopsy, a sample of lung tissue is taken and examined with a thin and lighted tube.

Idiopathic Pulmonary Fibrosis Treatment

There is no definitive treatment for idiopathic pulmonary fibrosis. The main purpose of the treatment of this disease is to keep the disease under control, minimize the symptoms of the disease and ensure that the patient’s quality of life is not impaired. Some of the treatment methods and preventive measures to be applied to slow down the progression of idiopathic pulmonary fibrosis and reduce the effects of the disease are as follows:

• quit smoking
• eating healthy
• exercising regularly
• Pirfenidone and nintedanib etc. prescribed by doctors to prevent the wound in the lung from worsening. regular use of medications containing active ingredients
• Using home oxygen masks
• Doing breathing exercises to make breathing easier, called pulmonary rehabilitation
• Taking protective measures and trying to keep immunity high to avoid catching flu or cold
• Getting the flu vaccine or pneumonia vaccine upon doctor’s recommendation