What is Pulmonary Fibrosis?
Pulmonary fibrosis is an end-stage change in a large class of lung diseases characterized by fibroblast proliferation and the accumulation of a large number of extracellular matrices, accompanied by inflammatory damage and tissue structure destruction. Abnormal (scar formation). The etiology of most patients with pulmonary fibrosis is unknown (idiopathic). This group of diseases is called idiopathic interstitial pneumonia (IIP) and is a large group of interstitial lung diseases. The most common type of disease in which idiopathic interstitial pneumonia (IIP) mainly manifests pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF), which is a serious disease that can cause progressive loss of lung function. Interstitial lung disease [1] . Pulmonary fibrosis severely affects the human respiratory function, manifested by dry cough, progressive dyspnea (inadequate gas consciousness), and with the worsening of the disease and lung damage, the patient's respiratory function continues to deteriorate. The incidence and mortality of idiopathic pulmonary fibrosis increase year by year. The average survival time after diagnosis is only 2.8 years. The mortality rate is higher than that of most tumors. It is called a "tumor-like disease" [2] .
Basic Information
- English name
- pulmonary fibrosis
- Visiting department
- Respiratory
- Multiple groups
- 40 to 50-year-old men, slightly more patients with previous smoking history
- Common causes
- Long-term smoking, environmental factors, chronic bronchitis and other lung diseases
- Common symptoms
- Chest tightness, dyspnea, etc.
Causes of pulmonary fibrosis
- When lung injury is caused by a variety of reasons, the interstitial tissue secretes collagen for repair. If it is over repaired, that is, fibroblasts proliferate excessively and extracellular matrix aggregates, lung fibrosis will form.
Clinical manifestations of pulmonary fibrosis
- Pulmonary fibrosis occurs more often in the 40-50 years old, and more often in men than women. Dyspnea is the most common symptom of pulmonary fibrosis. With mild pulmonary fibrosis, dyspnea often occurs during strenuous activity and is therefore often overlooked or misdiagnosed as another disease. As pulmonary fibrosis progresses, dyspnea also occurs at rest, and patients with severe pulmonary fibrosis may experience progressive dyspnea. Other symptoms are dry cough and weakness. Some patients have clubbing fingers and cyanosis. The severe consequences of pulmonary fibrosis lead to changes in the structure of normal lung tissue and loss of function. When a large number of fibrotic tissues that do not have gas exchange function replace the alveoli, the oxygen cannot enter the blood. Patients have difficulty breathing, hypoxia, acidosis, and loss of labor, and severe cases can eventually result in death.
Pulmonary fibrosis test
- Check-up
- Progressive shortness of breath, dry cough, wet snoring or twisting sounds in the lungs.
- 2.X-ray inspection
- Although there are early breathing difficulties, X-ray chest radiographs may be basically normal; diffuse reticular or nodular shadows appear in the middle and lower lungs in the middle and late stages, and occasionally pleural effusion, thickening or calcification.
- 3. Laboratory inspection
- It can be seen that the erythrocyte sedimentation rate increases, and generally has no special significance.
- 4. Lung function test
- Visible reduction in lung volume, reduced diffuse function, and hypoxemia.
- 5. Lung tissue biopsy
- Can provide pathological basis.
Pulmonary fibrosis diagnosis
- Diagnosis can be confirmed based on clinical manifestations, imaging, and pulmonary function tests.
Pulmonary fibrosis treatment
- Drug treatment
- (1) Pirfenidone:
- Pirfenidone is two of the "Recommended Guidelines for the Clinical Treatment of Idiopathic Pulmonary Fibrosis" jointly issued by the American Thoracic Society / European Respiratory Association / Japanese Chest Association / Latin American Chest Association in 2015. It is one of the drugs, and it is also the first drug approved for idiopathic pulmonary fibrosis (i.e. pulmonary fibrosis of unknown etiology, IPF for short) in the world. After 52 weeks of continuous treatment with pirfenidone, IPF patients can reduce the decline of lung function indicators such as FVC and DLCO, prolong progression-free survival (PFS), and reduce the risk of death.
- (2) Nidanib: Nidanib is in the "Recommended Guidelines for Clinical Treatment of Idiopathic Pulmonary Fibrosis" jointly issued by the American Thoracic Society / European Respiratory Association / Japanese Chest Association / Latin American Chest Association in 2015 One of the two drugs with the highest recommendation level (conditional recommendation).
- 2. Non-drug treatment
- oxygen therapy; mechanical ventilation; lung rehabilitation; lung transplantation.