About the disease
Fibrosing alveolitis is a condition that causes the alveoli to become thickened and inflamed. Alveoli are the tiny sacs in the lungs that are responsible for storing air and exchanges gases. Fibrosing alveolitis can result in the scarring of lung tissue, which is the most dangerous complication of this condition. Fibrosing alveolitis is most common in people over 60. It develops gradually and may take months or even years in certain cases for the first symptoms to manifest themselves. Nonetheless, in acute fibrosing alveolitis the disease can progress very rapidly and lead to scarring of the lung tissue in a matter of days.
Fibrosing alveolitis is often caused by autoimmune diseases, when the immune system starts to attack its own cells. The most common autoimmune diseases that can lead to the progression of fibrosing alveolitis, are rheumatoid arthritis and systemic lupus. Radiation therapy can also affect the lungs and lead to inflammation of the alveoli. Additionally, long-term smoking and tobacco consumption can lead to the alveoli becoming inflamed. Nonetheless, the exact cause of fibrosing alveolitis is unknown and scientists are still working on finding it out. In people with no autoimmune diseases or other underlying condition the disease is called idiopathic pulmonary fibrosis, meaning that the origin of alveolitis is not known.
Usually it is very difficult to stop the progression of this disease, but it is possible to do so in the early stages of fibrosing alveolitis. The disease can be fatal if decrease in lung function continues to progress. Also, clubbing of fingers can start to develop.
Symptoms
- Pain in the lungs
- Shortness of breath
- Finger clubbing
- Dry cough
- Crackling sound in the chest during coughing
Diagnosis
- During a general examination, the doctor will use a stethoscope to listen to the patient's breathing, to establish whether they have any difficulty in breathing and to listen out for any crackling sounds. The doctor will also examine the patient’s fingers for signs of clubbing.
- A blood test can determine whether there is a deficiency of oxygen in the blood, which can indicate fibrosing alveolitis.
- A lung function test can determine whether the patient has dyspnea, which is a chronic shortness of breath. It can also determine whether there is impaired gas exchange which would indicate that there is a problem with alveoli.
- An x-ray of the lungs will determine whether there is any scarring of the lung tissue.
Treatment
- The main goal of conservative treatment for this disease is to stop it from progressing and to alleviate its symptoms. Special antioxidants are prescribed, to slow down the progression of the disease. Drugs such as pirfenidone can reduce the risk of progression by 30%.
- Oxygen therapy can be prescribed if the patient suffers from shortness of breath.
- Lung transplantation is the most effective treatment for fibrosing alveolitis. It is recommended for patients whose lungs are scarred beyond repair but who are healthy enough to withstand the stress of surgery. By surgically removing the patient’s lung and transplanting a healthy one from a donor, the mortality rate reduces by 75%.
Authors: Dr. Vadim Zhiliuk, Dr. Sergey Pashchenko
