Idiopathic pulmonary fibrosis is the condition, where the lung tissues become stiff and thick due to scarring. In other words, it is the condition, where scar tissues develop in the lung and make it rigid and stiff. This in turn, makes it increasingly difficult to breathe properly. The affected lung loses its ability to deliver oxygen to the bloodstream.
The scarring of the lungs can be associated with repetitive injury or damage to the lung tissues, which can be caused by certain lung diseases. However, in many instances, physicians fail to determine the exact cause of this condition. This type of pulmonary fibrosis, where the cause is not known, is referred to as 'idiopathic pulmonary fibrosis'.
The scarring or fibrosis of the lung is generally caused by an extensive damage to the lung tissues and the tiny air sacs, known as alveoli. The scarring results when the body tries to heal the damaged tissues by replacing them with scar tissues. Even after conducting many studies and research, scientists have not been able to ascertain the exact underlying causes of idiopathic pulmonary fibrosis.
However, a number of factors are believed to contribute towards the development of this condition. Some such risk factors are, smoking, prolonged exposure to asbestos, silica, beryllium, hard metal dust, and organic dust, lung infections caused by bacteria and viruses, such as pneumonia and tuberculosis, acid reflux disease, and diseases of the connective tissues like scleroderma, sarcoidosis, rheumatoid arthritis, and systemic lupus erythematosus. Currently, genetics is also suspected to be a predisposing factor for this condition.
The symptoms of this condition are not very specific, as they can be similar to the symptoms of several other lung diseases. The most common symptom observed in patients is shortness of breath, especially on exertion, i.e., while doing a physical activity. The other symptoms of this condition are, a chronic and dry hacking cough, chest pain, fatigue and weakness, muscle and joint pain, clubbing or abnormal enlargement of the bases of the toes and fingernails, and a crackling sound in the lungs while breathing that can be heard with the help of a stethoscope.
It is quite difficult to diagnose this condition, as the signs and symptoms can become evident only when the lungs have been damaged to a great extent. The diagnosis of the disease involves a thorough evaluation of the medical history of the patient, followed by physical examinations. During a physical examination, abnormal breath sounds can be heard with the help of a stethoscope.
Moreover, the fingernails may appear bluish due to the lack of enough oxygen. The bases of the fingernails can also enlarge. However, a confirmed diagnosis typically requires a few other tests and examinations, like a chest X-ray, CT scan of the chest, pulmonary function test, measurement of the blood oxygen level, bronchoscopy, and lung biopsy.
The damage caused by the development of scar tissues in the lungs is not reversible. So, physicians basically aim at alleviating the symptoms and slowing down the progression of the disease. Some individuals can get relief by using certain medications like corticosteroids and cytotoxic drugs. Other treatment options include, oxygen therapy for people with low blood oxygen levels, and lung rehabilitation that can help maintain exercise tolerance. Lung transplantation is another treatment option, which is usually considered in the case of advanced pulmonary fibrosis.
Disclaimer: This HealthHearty article is for informative purposes only, and should not be replaced for the advice of a medical professional.