Having trouble breathing and dry cough, but can’t find the cause? Learn more about this disease!

Author: Fang Baomin, Chief Physician, Beijing Hospital

Reviewer: Huang Yuqing, Chief Physician, Beijing Haidian Hospital (Peking University Third Hospital Haidian Branch)

The lungs of a normal human body are like an inverted tree, with many alveoli hanging on its branches like bunches of grapes. It is a very elastic sponge-like organ, which is able to maintain the normal respiratory function of the human body.

Fibrosis occurs in the lung substance, mainly in the alveoli and alveolar septa, which widens the septa between the alveoli. After the widening, it is difficult for the breathed oxygen to enter the blood vessels, so the human body will experience difficulty breathing due to hypoxia.

If the cause of this type of pulmonary fibrosis cannot be found, we call it idiopathic pulmonary fibrosis.

1. What are the common symptoms and signs of idiopathic pulmonary fibrosis?

One of the main symptoms of idiopathic pulmonary fibrosis is shortness of breath or dyspnea during early activities. As the disease progresses, dyspnea becomes more and more obvious, and may be more obvious in a quiet state. Patients often come to the doctor for treatment at this time.

In addition to the common symptom of difficulty breathing, another accompanying symptom is coughing. Coughing is also a common symptom of these patients, mainly dry cough without much sputum.

Both symptoms are relatively common, occurring in more than 90% of patients, but neither is specific.

Figure 1 Original copyright image, no permission to reprint

If you do a physical examination, you will find a very typical or common sign called Velcro rales. These rales sound like the sound of pulling a nylon zipper, and they can be helpful in detecting the early stages of idiopathic pulmonary fibrosis.

Although it is not so specific, it is still very meaningful for paying attention to diseases such as interstitial lung disease or pulmonary fibrosis at an early stage and then making further diagnosis. Therefore, when doctors examine the patient, they will also pay special attention to whether the patient has Velcro rales.

In addition to Velcro rales, there is also clubbing. Patients sometimes say that their fingers have become as big as mallets, which is medically known as clubbing. This is also a common sign of idiopathic pulmonary fibrosis, but it is not specific. Some patients with bronchiectasis and lung cancer may also have signs of clubbing.

2. What are the treatments for idiopathic pulmonary fibrosis?

For early treatment, there are some anti-fibrosis drugs, one is pirfenidone and the other is nintedanib. These two drugs can delay the decline of lung function in these patients to a certain extent, but they cannot reverse it.

Figure 2 Original copyright image, no permission to reprint

During medication, attention should be paid to monitoring adverse reactions, as well as usage and dosage. For example, pirfenidone is recommended to be taken with meals, and patients are also advised to avoid direct sunlight to reduce photosensitivity reactions. For example, nintedanib may have some gastrointestinal reactions and abnormal liver function, which must be explained clearly to the patient.

In addition to inhibiting the further aggravation of pulmonary fibrosis, it is also necessary to actively deal with high-risk factors that cause or aggravate pulmonary fibrosis, such as smoking, keeping pets, gastroesophageal reflux, etc.

In addition, oxygen therapy should be performed when hypoxia occurs. During this process, rehabilitation should be carried out continuously.

If the patient enters the terminal stage and is estimated to survive less than two years, and follow-up observations reveal that the patient will also experience respiratory failure in a quiet state, affecting the quality of life, or has obvious pulmonary hypertension, then lung transplantation should be recommended. Of course, an assessment must be conducted before lung transplantation, and the next step of treatment can only be carried out if it is appropriate.

For some patients whose transplants go well, many can fully recover to a normal life or work, but they must take some anti-rejection drugs for a long time.

3. What should patients with idiopathic pulmonary fibrosis pay attention to in their daily lives?

The first step is to remove risk factors, such as quitting smoking, and then remove other factors that may trigger aggravation.

Another thing is the treatment of complications. For example, primary diseases such as gastroesophageal reflux may aggravate the degree of pulmonary fibrosis and should also be actively treated.

Another thing is to be able to intervene early and conduct regular monitoring, especially monitoring of lung function, to keep lung function in a relatively stable state.

Another thing is that patients can actively participate in clinical trials of new drugs, which may have a certain effect on the recovery of the disease. Participating in clinical trials can also establish a very good partnership between doctors and patients. Clinical trials are often conducted by more experienced doctors, who may give patients better guidance, communicate with patients, and increase patients' confidence in overcoming the disease.