PULMONARY FIBROSIS
Pulmonary fibrosis refers to a condition where scars are formed in the lung tissues.
- The scarring associated with the condition leads to thickening and stiffening of the walls and results in reduced oxygen supply in the blood.
- As the condition worsens, the patients suffer from perpetual shortness of breath.
- The course and symptoms may vary from person to person, while some become very ill quickly with severe diseases, others experience symptoms that worsen gradually, over months or years.
- Over time the condition can lead to respiratory failure, heart failure, lung cancer and other lung complications.
- Although the condition has been diagnosed in infants and children, it is more common in middle-aged and older adults.
Causes
Many factors are responsible for the scarring associated with the condition. However, in some cases, the doctor is unable to pinpoint the exact cause of the condition. When no cause is found, it is termed idiopathic pulmonary fibrosis.
Factors include:
Long-term exposure to a number of toxins and pollutants in the environment and workplace can contribute to the condition. Such as:
- Silica dust
- Coal dust
- Asbestos fibers
- Bird and animal droppings
- Certain gases
Radiation treatment for breast or chest cancer. The severity of the damage from the radiation depends on the period of time the lung was exposed to such radiation, the total amount of radiation administered, or the presence of any underlying lung disease.
Medications such as:
- Chemotherapy drugs such as methotrexate designed to kill cancer cell
- Anti-inflammatory drugs such as rituximab (Rituxan)
- Heart medications such as amiodarone ( Cordarone, Pacerone)
- Certain antibiotics such as nitrofurantoin ( Macrobid, Macrodantin)
Medical conditions such as:
- Pneumonia
- Sarcoidosis
- Rheumatoid arthritis
- Scleroderma
- Polymyositis
- And others
Infections
The certain risk for can increase your chance of getting the condition, this may include:
- Certain occupations such as coal minerals, sandblasters, and ship workers are at higher risk.
- Cigarette smoking
- Some type of pulmonary fibrosis run in families, and genetic factors may be a component
SYMPTOMS
Symptoms may include:
- Shortness of breath
- Fatigue
- Chronic, dry and hacking cough
- Unexplained weight loss
- Chest discomfort
- Muscle and joint aches
- Clubbing- curving of the fingernails or toes
DIAGNOSIS AND TREATMENT
To make a diagnosis, the doctor will review your family and medical history, discuss your symptoms and perform a physical examination.
The following test may be performed,
- Imaging tests such as chest x-ray, CT scan, echocardiogram
- Lung function tests such as pulmonary function testing, pulse oximetry, arterial blood gas test
- Lung biopsy
- Blood tests to look for autoimmune diseases, infections.
TREATMENT
Lung damage brought about by pulmonary fibrosis cannot be repaired, but medications and therapies can help with symptoms temporarily, slow down the disease’s progression and help with the quality of life.
The appropriate treatment options are determined by the severity of the condition.
Medications such as pirfenidone (Esbriet) and nintedanib (Ofev) may help slow down progression and is approved by the FDA.
Oxygen therapy to make breathing and exercise easier, reduce blood pressure on the heart, improve sleep and general wellbeing.
Pulmonary rehabilitation that focuses on physical exercise to improve endurance, nutritional counseling, counseling, and support.
Lung transplantation may be considered for some patients