Pulmonary fibrosis is a chronic disease characterized by deposition of connective tissue in the lungs and ultimately leading to airflow limitation. Pulmonary fibrosis occurs in various diseases and is manifested mainly by progressive dyspnea. Diagnosis of pulmonary fibrosis is based on clinical examination and laboratory investigations, but certainty diagnosis is put on histopathological examination.
Diagnosing pulmonary fibrosis can be extremely difficult because the difference between idiopathic and non-idiopathic pulmonary fibrosis is not always clear and the classifications for both diseases have created confusion. In addition, other diseases, including chronic obstructive pulmonary disease, asthma and heart failure can mimic pulmonary fibrosis, so doctors need to exclude these diseases before diagnosis.
Medical history, physical examination and chest radiography are not sufficient to diagnose pulmonary fibrosis, but they may exclude other diseases. In conclusion, these investigations which may be required, are:
- |e16b67c203e7a5a25ac285cdfd054abd| – this will provide information about the scar tissue, typical for pulmonary fibrosis, and is useful to determine the stage of the disease and treatment. In some cases, X-ray can be normal and further tests will be needed to explain the presence of dyspnea, for example.
- |fdeb90c3df14c1dacec3e3e3e4be059c| – this provides clear and detailed images of the lungs, unlike conventional scans or chest X-rays.
- |f722b042ff9a59997218af95da4e1464| – establish the lung function: the amount of air stored in the lungs.
- |6bbcf2b82d1db8e7778a960ab473a506| – this simple test is performed through a small device that is placed on one of the fingers and is designed to measure blood oxygen saturation. Oximetry is the simplest way of monitoring the disease, sometimes more accurate than chest X-ray.
- |50f58a1f8e63980ac3b752e448165c7c| – is performed on the treadmill or on a stationary bike, and is designed to monitor lung function during physical activity.
Often, pulmonary fibrosis can be diagnosed accurately by sampling a small sample of lung tissue (biopsy) analyzed in the laboratory. The tissue sample can be obtained as follows:
- |56dc424b788c81d934cb1ac6dbf5ec94| – the specialist removes a very small tissue sample, usually no bigger than the tip of a needle – with a small, flexible tube (bronchoscope), which is inserted through the mouth or nose into the lungs. In general, the risks are minor, most often sore throat and hoarseness are temporary.
- |8fae50d3a90741c5946253b3e86bd284| – the doctor injects salt water (saline) through a bronchoscope into a section of the lung and will immediately vacuum. The solution contains cells aspirated from alveolar sacs. Although the samples obtained by bronchoalveolar lavage are bigger than those from the first procedure, this procedure not always provide sufficient information for the diagnosis of pulmonary fibrosis.
- |e839a9d4ee1168d92646263bbfb88336| – this procedure is more invasive than others, and is the only way to get a tissue sample large enough for the diagnosis.
Lung scarring can not be cured and no treatment has not proven to be effective in stopping the evolution of pulmonary fibrosis. Some treatments can temporarily reverse the disease and will improve the quality of life.
Patients with pulmonary fibrosis are initially treated with corticosteroids (prednisone), sometimes in combination with other drugs that stimulate the immune system (methotrexate or cyclosporine). However, none of these combinations has not proved very effective at all.
Lung transplantation may be an option of last resort for young people with severe pulmonary fibrosis who have not benefited from other treatment options. To fit the conditions necessary for transplant should stop smoking (if they smoke) and to be healthy enough to cope with surgery and post-transplant treatment, and be willing and able to undergo to the necessary medical rehabilitation program after transplant and to be patient and to be supported emotionally as long as are waiting for a donor. This last specification is important because waiting lists for organ donation are very long.
Other treatments for pulmonary fibrosis focuses on improving quality of life. These include:
- Oxygen therapy – Oxygen does not cure lung lesions, but contribute to the process of respiration, prevent and reduce complications in the presence of a low oxygen level in blood, improves sleep quality and provides better overall body condition. Oxygen therapy reduces symptoms of right heart failure.
- Pulmonary rehabilitation – This is a formal program for people with chronic lung diseases, including medical management. The purpose of pulmonary rehabilitation is not only to treat disease or relieve symptoms, but will help people with pulmonary fibrosis to live a satisfactory life. Pulmonary rehabilitation programs focus on breathing techniques, effective learning, on education, emotional support and nutritional counseling.
Active involvement in treatment and maintaining a good general health are the main goals for the patient with pulmonary fibrosis. It is important to:
- |aa8ea98ff4b5c0eaed69d70a8d49e7ec| – The first thing for a person diagnosed with pulmonary fibrosis is to quit smoking. There are several programs that use a variety of techniques that have proved useful in this regard. And because passive smoking is as harmful to the lungs, do not let others smoke around you.
- |7394d1c2f6a6c64bb9c6742001f44ccf| – Physical exercise is a double-edged sword for people diagnosed with lung disease. Doctors recommend patients to practice breathing exercises for short term, in order to keep a good lung function and to reduce stress. Mild physical exercise can help. If you are an active person talk to your doctor about a exercise program that could be effective for you.
- |c3590f9e951a1e8e0f8f845de76e6ef3| – Patients with lung disease may lose weight because they can manifest discomfort during eating, which leads to consuming smaller amounts of food, but also because a lot of energy is consumed by respiration effort. It is recommended a nutrient rich diet that contains essential fats, vitamins and minerals.
- |e2844bfe8096582767109eb454001e8d| – Are recommend at least 8 hours of sleep per night for the immune system to be stimulated.
- |f9c688eeb3ae2ef89410187ace15c872| – is recommended: a break of at least three hours between meals, eat small meals and avoid any foods that trigger the symptoms.