Choose the content to read

• What causes pulmonary fibrosis?
• What are the symptoms of pulmonary fibrosis?
• How is pulmonary fibrosis diagnosed?
• How is pulmonary fibrosis treated?
• Lifestyle modification and home remedies
• FAQ

Pulmonary Fibrosis

Pulmonary fibrosis is a respiratory disorder with scarring of lung tissues between the air sacs (alveoli). The damaged tissues become stiff and thickened, leading to compromised lung function and impaired oxygen transfer into the bloodstream. Various factors can trigger lung damage, including prolonged exposure to specific toxins, certain medical conditions, radiation therapy, and certain medications.

Regrettably, the lung damage associated with pulmonary fibrosis is irreversible, but there are treatment options available to manage symptoms and enhance overall quality of life. Medications and therapies can provide relief for some individuals. In some cases, lung transplantation may be a viable consideration for improving the condition.

What causes pulmonary fibrosis?

• Long-term exposure to toxins and pollutants such as coal dust, grain dust, asbestos fibers, silica dust, and animal droppings in the environment or workplace
• Lung cancer or breast cancer radiotherapy. The extent of the harm depends on how much of your lungs received the radiation, the dosage of the radiation, and whether there is concurrent chemotherapy. 
• Chemotherapy drugs, such as bleomycin, methotrexate, and cyclophosphamide
• Heart arrhythmia medication such as amiodarone.
• Antibiotics such as nitrofurantoin. 
• Anti-inflammatory medicines, such as rituximab or sulfasalazine
• Medical conditions, such as dermatomyositis, mixed connective tissue disease, rheumatoid arthritis, pneumonia, polymyositis, sarcoidosis, scleroderma, and systemic lupus erythematosus.

Certain types of pulmonary fibrosis, called idiopathic pulmonary fibrosis, usually affect seniors, about 70 to 75 years old, and may run in the family; the causes are not clear. According to research, GERD may also be a factor in the progression of pulmonary fibrosis, but more study about its association is required.

What are the symptoms of pulmonary fibrosis?

• Rapid, shallow breathing.
• Persistent dry cough. 
• Extreme daytime tiredness.
• Shortness of breath during or after exercise.
• Unexplained weight loss.
• If symptoms progress, you may develop clubbed fingers or toes and cyanosis due to low oxygen saturation.

Risk factors

• Age. The risk of pulmonary fibrosis is higher in the middle-aged and elderly.
• Sex. Males are at higher risk than females.
• Smoking. Smokers, former smokers, or people with emphysema have increased risk. 
• Exposure to pollutants from workplaces such as mining, farming, or construction 
• Cancer treatments, i.e., radiotherapy and chemotherapy.
• Genetic factors. Some types of pulmonary fibrosis can be hereditary and passed on through generations.

How is pulmonary fibrosis diagnosed?

• History taking and physical exam
  • Your doctor will review your personal and family medical history, ask about signs and symptoms, and listen to your lungs while you breathe.
• Blood tests
  • Blood tests can help assess your liver and kidney functions and rule out other conditions.
• Imaging tests
  • Chest X-ray is a screening test to see if there are any pulmonary abnormalities.
  • Computerized tomography (CT) scans can determine the extent of lung damage. It can help monitor your condition and evaluate if your symptoms respond to treatment.
  • An echocardiogram can assess the pressure in your right ventricle in patients with complications related to pulmonary fibrosis.
• Lung function tests
  • Pulmonary function testing, such as spirometry to measure the diffusing capacity and the volume of your lung
  • Pulse oximetry to noninvasively measure the amount of oxygen circulating in your blood with a device placed on the tip of your fingers.
  • Exercise stress test to monitor your cardiac function.
  • Arterial blood gases
• Biopsy
  • Bronchoscopy.
  • Surgical biopsy with video-assisted thoracoscopic surgery (VATS) or thoracotomy.

How is pulmonary fibrosis treated?

Pulmonary fibrosis is permanent, but early diagnosis and treatment can improve and prolong your lung function.

• Medications such as pirfenidone and nintedanib are anti-fibrotic agents. They help slow down the rate of fibrosis or scarring in the lungs.
• Oxygen therapy can raise your blood oxygen levels. It can decrease shortness of breath and increase your stamina.
• Pulmonary rehabilitation to reduce fatigue and improve the quality of life of patients
• Lung transplant

Complications

Pulmonary hypertension can occur because scars in your lungs may decrease the size of arteries and capillaries, leading to raised lung vascular resistance and increased pressure in the right ventricle. Certain types of pulmonary hypertension can be life-threatening.

• Cor pulmonale happens when the right ventricle has to pump against high pulmonary artery blood pressure for a long duration, leading to right heart failure.  
• Respiratory failure due to dangerously low blood oxygen levels in the final stage of this lung disease  
• Lung infections.
• Pulmonary embolism.
• Collapsed lungs or pneumothorax
• Lung Cancer. 

Lifestyle modification and home remedies

• Quit smoking. 
• Eat a nutritionally rich diet with adequate calories because people with pulmonary fibrosis tend to lose weight. Try six smaller meals instead of three big meals during the day.  
• Regularly exercise to keep your lungs healthy and relieve stress.
• Have adequate rest. 
• Get vaccinated to minimize the risk of respiratory infections, such as influenza or pneumonia.
• Strictly follow your doctor’s instructions and treatment plan. 

Preparation before the doctor’s appointment

Write down answers to questions that your doctor may ask you.

• When did you first notice your symptoms?
• What symptoms do you have?
• Do you have any preexisting conditions, such as arthritis?
• Have you taken any medications or dietary supplements in the past five years?
• Have you been exposed to excessive dust in your workplace?
• Do you regularly smoke?
• Does anyone in your family have chronic lung disease?
• Have you had radiotherapy or chemotherapy?

Bring along a friend or family member to help you remember all the information and be your emotional support because pulmonary fibrosis is a severe and complex disease.

FAQ

• What are the differences between pulmonary fibrosis and chronic obstructive pulmonary disease?
  Both chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis are severe and progressive diseases that cause breathing problems. 
  Pulmonary fibrosis occurs when scar tissues build up in the lungs, making breathing difficult. The root cause is usually unknown. 
  In COPD, your lung tissues become irritated and inflamed with the destruction of alveoli, causing shortness of breath and airways clogged with phlegm. The predominant cause is usually smoking. Asthma or excessive exposure to pollutants can increase the risk of COPD with emphysema and bronchitis.

Advice by MedPark’s doctor

People with pulmonary fibrosis need regular follow-ups with a doctor to monitor their illness and course of treatment. Suitable combinations of treatments can help improve lung function and quality of life.