Pulmonary Fibrosis: Causes, Symptoms and Treatment

Pulmonary fibrosis is the disease in which lung tissue becomes thick and stiff as compared to its normal elastic form. The tissue basically becomes scarred dead material hampering the basic functions of the lung that is the exchange of gases between blood and the environment. Patients generally have difficulty in breathing and recurrent cough. In most of cases, the cause of fibrosis can not be determined and in medical terms, it is called as idiopathic pulmonary fibrosis. Over more than 50 thousand of cases are diagnosed each year.

Types of Pulmonary Fibrosis

There are over 210 types of Pulmonary Fibrosis and in most of the types, the cause of fibrosis is not known. The common types are

• Idiopathic – when the cause is not known.
• Exposure to hazardous material

Symptoms of Pulmonary Fibrosis

In most people suffering from PF symptoms develop between 50 to 70 years of age.

• Shortness of breath – Patients have difficulty in breathing, they tend to breathe forcefully.
• Dry cough
• Unwanted weight loss
• Joint and muscle pain
• Swelling of fingertips and toes known as clubbing

Causes of Pulmonary Fibrosis

Occupation related or environmental pollutants

• Coal dust
• Silica dust
• Asbestos
• Bird droppings
• Cotton, flax, hemp dust

Associated with other diseases

• Sarcoidosis
• Scleroderma
• Rheumatoid arthritis
• SLE (systemic lupus erythematosus)

Drugs associated

• Anti-inflammatory
• Anti- cancerous drug

Diagnosis of Pulmonary Fibrosis

• Physical examination: The physician will listen to both of your lungs with the help of a stethoscope, the presence of crackling or bubbling sound may indicate fibrosis.
• Imaging techniques:

            Chest X-ray: in the early stages of pulmonary fibrosis the chest x-ray is normal most of the time so a CT scan is required.

            CT scan: A high-resolution CT scan is done to find out the tissue architecture of the lung such that a 3-dimensional structure is created.

            Echocardiogram: It is done for patients with heart complications associated with pulmonary fibrosis.

• Pulmonary function test: These tests basically find out how efficient or hard our lungs are working to move the air in or out.
• Lung tissue sample: it is also called lung biopsy which reveals the actual process going inside your lungs.

Treatment of Pulmonary Fibrosis

The lung tissue which has undergone fibrosis can’t be reversed back to normal. The changes are permanent. Every person’s condition is unique because there is no standard clinical course.

Treatment comprises of medication, non-medication therapy (like exercise), oxygen therapy, and at last the lung transplantation.

• Medications: It includes antifibrotic drugs such as nintedanib, pirfenidone anti-inflammatory drugs like corticosteroids, mycophenolic acid, azathioprine.
• Oxygen therapy: Generally advised when the oxygen level of blood is below 88 %.
• Pulmonary rehabilitation: By the help of breathing exercises, stress management, proper counseling the quality of life of such people can be improved.
• Lung transplantation: There are some patients whose conditions become so worse that they can’t survive enough without whole lung transplantation.

Prognosis of Pulmonary Fibrosis

The prognosis of pulmonary fibrosis is not good as there is no effective treatment that can stop the progression of the disease but the progression can be delayed and that varies greatly from person to person because some people have a progressively deteriorating course despite being treated continuously.