What is idiopathic pulmonary fibrosis and what are the dangers

Idiopathic pulmonary fibrosis is a new type of lung disease. Because it is acute and idiopathic, the disease comes on suddenly and is relatively rare, so it is easily ignored and misdiagnosed. Many patients with idiopathic pulmonary fibrosis only realize that they have idiopathic pulmonary fibrosis after months of suffering. Idiopathic pulmonary fibrosis poses certain dangers and requires timely treatment.

What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis, abbreviated as IPF, is a chronic, progressive, irreversible and fatal lung disease. "Idiopathic" means the cause is unknown, which means the exact cause is unknown. Unexplained antigen stimulation causes repeated damage to lung tissue. In order to repair the damaged lung tissue, the production of collagen and other substances increases, causing the lung interstitium to thicken, leading to lung fibrosis. In layman's terms, the normal tissue in the lungs gradually degenerates and loses its original function of supplying oxygen to other tissues and organs, and this transformation is irreversible. Clinically, the disease is mainly manifested by dyspnea, dry cough, and limited movement due to hypoxia.

The disease occurs all over the world, regardless of race. It is more common among the elderly, with a slightly higher incidence in men than in women. Its natural course varies greatly and is unpredictable. Although it is a rare disease, its incidence has shown an increasing trend in recent years. In the United States, the number of people suffering from idiopathic pulmonary fibrosis is 132,000-200,000, with approximately 50,000 new cases diagnosed each year and 40,000 deaths. The estimated number of cases in the EU is between 37,000 and 40,000. Among them, the UK confirms more than 5,000 new cases each year. It is important to note that, according to relevant speculation, the number of confirmed cases of IPF is still increasing. This may be related to people's longer life expectancy, clinicians' increased awareness of IPF and advances in diagnostic technology.

There is no effective drug at present, but various countries are conducting relevant clinical trials. I believe that effective drugs will be available soon. We should actively cooperate with the treatment and do not give up treatment.

How terrible is idiopathic pulmonary fibrosis?

The disease progression of IPF manifests itself differently in different patients. In some people, the disease and symptoms remain relatively stable for years, whereas in others the disease progresses rapidly. One of the characteristics of IPF is that the course of the disease can have unexpected jumps (sudden deterioration), which is medically known as "acute exacerbation". Signs of worsening symptoms include intense shortness of breath, severe coughing, and fatigue. The prognosis of IPF is poor, with a mortality rate higher than some tumors and second only to lung cancer among lung diseases. The median survival from diagnosis to death is 2 to 3 years, and its natural course varies greatly and is unpredictable.

Is there any medicine that can treat it?

Domestic pirfenidone (Acerui) has no special adverse reactions and shortness of breath has improved compared to before. Pulmonary function FVC (forced expiratory volume) was 79.4%, DLCO (diffusing capacity of carbon monoxide) was 49%, blood gas analysis PaO2 (oxygen partial pressure) was 73 mmHg, PaCO2 (carbon dioxide partial pressure) was 37 mmHg. The most common adverse reactions of pirfenidone were gastrointestinal reactions, skin diseases and dizziness, which were usually mild to moderate in severity. Most adverse events could be relieved after dose reduction or drug discontinuation. Clinically, it has been confirmed that although the adverse reactions of pirfenidone are common, they can be gradually improved under the guidance of a doctor by changing the way of taking the medicine and adjusting the dosage. Long-term efficacy of pirfenidone in inhibiting the progression of pulmonary fibrosis and delaying disease progression.