What is the pathophysiology of pulmonary fibrosis?
What is the pathophysiology of pulmonary fibrosis?
Currently, pulmonary fibrosis is regarded as a disease caused by repeated subclinical injury leading to epithelial damage and subsequent destruction of the alveolar-capillary basement membrane. This process initiates the infiltration of fibrotic cells and the activation of (myo)fibroblasts.
What is interstitial pulmonary fibrosis?
Interstitial lung disease refers to a group of about 100 chronic lung disorders characterized by inflammation and scarring that make it hard for the lungs to get enough oxygen. The scarring is called pulmonary fibrosis. The symptoms and course of these diseases may vary from person to person.
Is interstitial pulmonary fibrosis the same as idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis belongs to a group of conditions called interstitial lung diseases (also known as ILD), which describes lung diseases that involve inflammation or scarring in the lung.
What is the most common cause of interstitial lung disease?
A bacteria called Mycoplasma pneumoniae is the most common cause. Idiopathic pulmonary fibrosis. This makes scar tissue grow in the interstitium.
What happens pulmonary fibrosis?
Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.
What are the cellular events in pulmonary fibrosis?
The pathologic findings in pulmonary fibrosis (excessive accumulation of ECM and remodeling of the lung architecture) are a consequence of disturbances in two physiologically balanced processes: proliferation and apoptosis of fibroblasts, and accumulation and breakdown of ECM.
What means interstitial?
Medical Definition of interstitial 1 : situated within but not restricted to or characteristic of a particular organ or tissue —used especially of fibrous tissue. 2 : affecting the interstitial tissues of an organ or part interstitial hepatitis.
What is the difference between COPD and interstitial lung disease?
In IPF, your lungs become scarred, stiff, and thick, and the progressive damage is not reversible. In COPD, the airways, which are branching tubes that carry air within the lungs, become narrow and damaged. This makes people feel out of breath and tired.
Is lung scarring always pulmonary fibrosis?
To break it down phonetically: pulmonary means lung and fibrosis means scar tissue, so basically the name pulmonary fibrosis translates to: scarring in the lungs. Beyond the problems scars present, pulmonary fibrosis impacts how you breathe and get oxygen into the blood.
What are the first signs of pulmonary fibrosis?
Signs and symptoms of pulmonary fibrosis may include:
- Shortness of breath (dyspnea)
- A dry cough.
- Fatigue.
- Unexplained weight loss.
- Aching muscles and joints.
- Widening and rounding of the tips of the fingers or toes (clubbing)
What is the life expectancy of someone with interstitial lung disease?
The average survival for people with this type is currently 3 to 5 years . It can be longer with certain medications and depending on its course. People with other types of interstitial lung disease, like sarcoidosis, can live much longer.
Can you reverse pulmonary fibrosis?
The lung scarring that occurs in pulmonary fibrosis can’t be reversed, and no current treatment has proved effective in stopping progression of the disease. Some treatments may improve symptoms temporarily or slow the disease’s progression. Others may help improve quality of life.
What are the stages of interstitial lung disease?
In the end stage of chronic interstitial lung disease, respiratory failure occurs when severely low blood oxygen levels along with rising pressures in the pulmonary arteries and the right ventricle cause heart failure. Raghu G, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis.
What is the prognosis for interstitial lung disease?
The prognosis for patients depends on how severe the condition is, and the cause of the ILD. Many people with mild ILD can function normally throughout their lives without treatment. Their condition can be stable (doesn’t get worse). For people with more severe cases of ILD, the disease can be progressive (gets worse over time).
