- Medical Editor: John P. Cunha, DO, FACOEP
Reviewed on 8/11/2022
- Symptoms
- What Are Symptoms of Scarred Lungs?
- Causes
- What Causes Scarred Lungs?
- Diagnosis/Tests
- How Are Scarred Lungs Diagnosed?
- Treatment
- What Is the Treatment for Scarred Lungs?
- Complications
- What Are Complications of Scarred Lungs?
- Guide
The average life expectancy for a person with scarred lungs used to be three to five years after diagnosis but with improvements in treatments, the prognosis may be better, depending on several factors.
The medical term for scarred lungs is pulmonary fibrosis (PF), which is an umbrella term for over 200 types of lung disorders. All these disorders belong to larger family of lung conditions called interstitial lung disease (ILD), in which there is inflammation and/or scarring of lung tissue.
According to The Pulmonary Fibrosis Foundation, there is no way to know how long a person with scarred lungs will live. The average life expectancy for a person with scarred lungs used to be three to five years after diagnosis, but improvements in treatments mean those estimates are now outdated.
Factors that influence a patient’s prognosis include:
- Early diagnosis
- Linked with better outcomes
- Getting proper treatment
- Though there is no cure for pulmonary fibrosis, anti-fibrotic treatments can slow lung scarring and disease progression
- Lung function
- Certain factors, such as rapid lung function decline over time and worsening shortness of breath are associated with a poorer prognosis
- Smoking
- One of the main risk factors for lung scarring
- Current and former smokers tend to have a worse prognosis
- Age, sex, and body mass index (BMI)
- Older people have a worse prognosis than younger people
- Pulmonary fibrosis is more common in males
- People with a lower BMI often have a worse outlook
What Are Symptoms of Scarred Lungs?
Symptoms of scarred lungs (pulmonary fibrosis) are progressive and gradually worsen over time. Symptoms of scarred lungs may include:
- Respiratory symptoms
- Shortness of breath (one of the most common symptoms)
- Fast, shallow breathing due to low blood oxygen levels
- In adults, this is usually more than 20 breaths per minute
- Persistent, dry, or hacking cough
- Other symptoms
- Significant fatigue that does not go away with rest
- Sleep issues such as sleep apnea
- Muscle and joint aches
- Swelling at the tips of the fingers and/or toes (digital clubbing)
- Loss of appetite
- Weight loss without trying
What Causes Scarred Lungs?
There are two main types of scarred lungs (pulmonary fibrosis): idiopathic pulmonary fibrosis (IPF) and familial pulmonary fibrosis (FPF). Familial pulmonary fibrosis is rare and defined when two people in the same biological family develop IPF and/or any other form of idiopathic interstitial pneumonia.
The most common form of the disease is idiopathic pulmonary fibrosis, in which there is no clear cause.
Risk factors that increase the risk of developing idiopathic pulmonary fibrosis include:
- Genetics
- Genes do not cause pulmonary fibrosis but they can make a person more susceptible to the disease
- Male sex
- Cigarette smoking
- Exposure to certain occupational and environmental hazards
- Asbestos fibers
- Silica dust
- Coal dust
- Hard metal dust
- Irritants such as cigarette smoke or animal droppings
- Certain medications and medical treatments
- Certain forms of radiation therapy
- Some forms of chemotherapy
- Antibiotics, such as nitrofurantoin (Furadantin, Macrodantin, Macrobid)
- Anti-inflammatory medications, such as sulfasalazine (Azulfidine, Azulfidine EN-tabs)
- Medications for heart conditions, such as amiodarone (Pacerone, Nexterone)
- The anti-seizure medication phenytoin (Phenytek, Dilantin Infatabs, Dilantin Kapseal)
- Other diseases
- Certain autoimmune diseases
- Rheumatoid arthritis
- Systemic scleroderma
- Sjögren’s syndrome
- Certain autoimmune diseases
- Viral infections
- COVID-19
- Certain muscle diseases
- Polymyositis
- Anti-synthetase syndrome
How Are Scarred Lungs Diagnosed?
Scarred lungs (pulmonary fibrosis) are diagnosed with a medical history and physical examination in which a doctor will listen to the lungs with a stethoscope for a characteristic “crackling” sound that can be a sign of scar tissue.
There is no single test to detect PF, but tests used to help confirm a diagnosis include:
- Imaging tests
- X-ray of the chest
- High-resolution computerized tomography (HRCT) scan
- Echocardiogram (“echo”)
- Lung function tests
- Spirometry
- Gas diffusion study or diffusing capacity of the lung for carbon monoxide (DLCO),
- Exercise test (also called a stress test)
- Blood tests
- To check oxygen levels in the blood
- Pulse oximetry
- Arterial blood gas test
- To evaluate the function of the liver and kidneys
- To rule out other possible conditions such as infections
- Measuring how much oxygen is circulating in the bloodstream
- To check oxygen levels in the blood
- Bronchoscopy
- Biopsy
What Is the Treatment for Scarred Lungs?
There is currently no cure for scarred lungs (pulmonary fibrosis) but there are medications and other treatments that can help improve patients’ quality of life, reduce symptoms, and slow the progression of the disease.
Medications used to treat pulmonary fibrosis include:
- Cyclophosphamide (Cytoxan)
- A chemotherapy drug used to help suppress inflammation
- Pirfenidone (Esbriet)
- Used to decrease inflammation
- Azathioprine (Imuran)
- An immunosuppressant used with other medications to prevent rejection of transplants
- Also is used to treat inflammatory diseases, connective tissue diseases, and autoimmune diseases when other medications and treatments do not work
- Mycophenolate mofetil (CellCept, Myfortic)
- An immunomodulatory drug that is used to prevent transplant rejection, and as an anti-inflammatory and an anti-proliferative drug
- Nintedanib (Ofev)
- An anti-fibrotic medicine that inhibits tyrosine kinase which helps slow the progression of IPF
- Omeprazole (Prilosec)
- A proton-pump inhibitor (PPI) that helps decreased stomach acid and is used to treat acid reflux which is common in people with scarred lungs
- Treprostinil (Tyvaso)
- An inhaled medication used to treat pulmonary hypertension associated with interstitial lung disease (including IPF)
- It helps improve blood circulation while keeping blood pressure low
Other treatments that can help patients who have scarred lungs include:
- Oxygen therapy
- To prevent breathlessness
- Pulmonary rehabilitation
- An education- and exercise-driven program that helps improve lung health and function in people with chronic lung diseases
- Palliative care
- To help improve quality of life by relieving symptoms
- Lung transplant
What Are Complications of Scarred Lungs?
Potential complications of scarred lungs (pulmonary fibrosis) include:
- Pneumothorax
- Partially or completely collapsed lung
- Pneumomediastinum (mediastinal emphysema)
- Air leaks out into the space between the lungs in the chest
- Pulmonary edema
- Fluid buildup in the lungs
- Pulmonary embolism (PE)
- A blood clot in the lungs
- Pulmonary hypertension
- Blood pressure in the blood vessels of the lungs is excessively high which puts strain on the heart and can lead to heart failure
From 
Lung Disease/COPD Resources
Reviewed on 8/11/2022
References
REFERENCES:
Image source: iStock Images
https://responsumhealth.com/pulmonary-fibrosis/prognosis/
https://pulmonaryfibrosisnews.com/
