Pulmonary Fibrosis

Pulmonary fibrosis is a disease of the lungs that can have many different causes. The lungs become stiff due to scarring. Breathing difficulties and reduced resilience are typical. Pulmonary fibrosis cannot be cured, only alleviated.

Pulmonary Fibrosis

What is pulmonary fibrosis?

According to Aviationopedia, pulmonary fibrosis is a chronic inflammation of the lungs, which usually starts with inflammation of the air sacs (alveoli). This inflammation leads to increased formation of connective tissue in the lungs, causing the lung tissue to become scarred and stiff.

This greatly reduces the elasticity and volume of the lungs, and the gas exchange in the lungs is severely impaired. Not only is breathing itself more difficult, the scarring also makes it more difficult for oxygen to get into the blood and the shortness of breath is increased.

The longer the pulmonary fibrosis lasts, the harder it is on the heart. If there is no cause for the pulmonary fibrosis, the doctor speaks of an idiopathic pulmonary fibrosis.

Causes

Pulmonary fibrosis can have different causes, but the body’s own collagen always forms uncontrollably. Collagen is almost inextensible and is the most important protein found throughout the human body. It is one of the most important components of human connective tissue and is therefore found primarily in bones, teeth, cartilage, tendons, ligaments and of course the skin.

The diseases that can cause pulmonary fibrosis include, for example, inflammatory processes. Circulatory disorders, various infections caused by viruses, bacteria or fungi, systemic diseases and toxins can also cause pulmonary fibrosis.

A very important cause in recent years was asbestos, which was used throughout the construction industry. Long-term inhalation of asbestos fibers has been shown to lead to inflammatory reactions and the development of pulmonary fibrosis. In half of the cases, however, the cause is also unknown.

Symptoms, Ailments & Signs

Lung fibrosis can go undetected for years. The first symptoms only appear once the organ has already been severely damaged. The signs of the disease include shortness of breath and a dry cough, with breathing difficulties initially only occurring during physical exertion. Only in the advanced stage do those affected suffer from shortness of breath even when at rest.

Accompanying this, an oppressive feeling of tightness can be noticed when inhaling. In individual cases, breathing is blocked and the lungs only open again after a few breaths. The difficulty in breathing eventually leads to an insufficient supply of oxygen to the body. This can be recognized by the blue-colored fingers and the so-called drumstick fingers, which are expressed by swollen finger ends.

Signs of the disease also include conspicuously curved fingernails and paleness. People suffering from pulmonary fibrosis suffer from infections of the respiratory tract and lungs with disproportionate frequency. In the worst case, it can lead to pneumonia, which is announced, among other things, by pain when breathing and an increasing feeling of illness.

In addition, fibrosis of the lungs can cause muscle and joint pain, exhaustion, fatigue, fever, loss of appetite and weight loss. In the advanced stage, pulmonary hypertension often develops, which can lead to heart damage and accumulation of fluid in the abdomen.

Diagnosis & History

Patients with pulmonary fibrosis often suffer from coughing, fatigue and severely limited exercise capacity. A first symptom is water retention in the lungs ( pulmonary edema ), as the walls of the alveoli are thickened. Gas exchange between blood and lungs is impeded.

Initially, shortness of breath occurs under stress, but in an advanced stage, shortness of breath also occurs at rest. The respiratory rate is increased because the patient has to inhale against resistance.

If the pulmonary fibrosis is more advanced, the skin also changes due to the lack of oxygen. The skin and mucous membranes turn bluish, the fingernails and toenails thicken like a flask, are thicker and rounder than normal fingernails or toenails (drumstick fingers) and bulge like a watch glass (watch glass nails). In general, these are signs of a prolonged lack of oxygen supply to the tissue.

The auscultatory findings (i.e. the stethoscope) can provide the first indication of pulmonary fibrosis. In addition to questioning the patient, a lung function test and X-rays are part of the examination. The diagnosis is confirmed by a lung reflection, in which the doctor takes samples of the lung tissue.

Complications

In the worst case, pulmonary fibrosis can lead to the death of the patient. With early treatment, however, the limitations and symptoms can be treated and resolved relatively well. In most cases, those affected suffer from shortness of breath and also from shortness of breath. The lungs can no longer expand in the usual way and thus no longer absorb enough oxygen.

As a result, those affected feel exhausted and tired. Reduced resilience continues to occur, so that patients no longer actively participate in their lives. A strong cough also occurs, which can lead to various limitations in the patient’s everyday life. In general, the quality of life of those affected is significantly restricted and reduced by pulmonary fibrosis.

Pulmonary fibrosis can be treated with a variety of medications. There are no further complications. However, this treatment does not always lead to a positive course of the disease. It is not uncommon for those affected to be dependent on a lung transplant in order to continue to survive. In many cases it is only possible to slow down the progression of pulmonary fibrosis.

When should you go to the doctor?

If you experience shortness of breath or breathing difficulties, you should consult a doctor. If you have a persistent cough or a dry cough, you need to see a doctor. The disease usually goes unnoticed at first, as the patient is symptom-free for a long time. As a result, the symptoms only appear in an advanced stage of the disease and this should then be classified as quickly as possible. If the person concerned suffers from anxiety due to restricted breathing, a doctor should be consulted.

If breathing is blocked or there are irregularities when breathing in, a doctor is needed. A doctor should be consulted if signs such as tiredness, weakness, exhaustion or rapid fatigue appear. If the usual services can no longer be provided, a comprehensive examination is advisable. Pain in the joints, bones or muscles is cause for concern. A decrease in body weight, the development of swelling or irregularities in the heart rhythm must be checked and clarified by a specialist.

If water retention develops, if there are abnormalities in the skin’s appearance or if apathy occurs, a doctor’s visit is necessary. Discoloration of the fingers, pallor of the skin or problems with blood circulation in the limbs are signs that should be discussed with a doctor.

Treatment & Therapy

At the current state of medical research, pulmonary fibrosis cannot be cured. Scarred tissue no longer reforms. Therefore, the main goal of therapy is to stop or at least slow down the spread of scarring and stiffening of the lungs.

If a toxin is the cause of the fibrosis, the patient must of course strictly avoid the triggering pollutant immediately.

When it comes to medicines, the doctor often prescribes cortisone preparations and immunosuppressants for inflammatory processes, i.e. medicines that inhibit the immune system and have a positive influence on inflammatory processes. Depending on the severity and stage of the disease, the administration of oxygen may also be necessary.

If pulmonary fibrosis is severe and drug treatment is no longer possible, lung transplantation is indicated.

It is difficult to predict the success of the therapy, since there are many different triggers for pulmonary fibrosis and the course of the disease can therefore vary greatly.

Outlook & Forecast

If pulmonary fibrosis has been diagnosed, no concrete statements can be made about a general prognosis. The reason for this is the fact that the course of the disease is not uniform and the clinical picture can express itself in many different ways.

In certain cases, pulmonary fibrosis may have little or no impact on the patient’s life expectancy. In other cases, the disease can also progress quickly. If it is pulmonary fibrosis that occurs without recognizable causes, the prognosis is usually poor.

In general, it can be said that pulmonary fibrosis cannot be cured. This means that the lung tissue that has been altered by scarring does not recover. Pulmonary fibrosis is classified as an extremely serious condition and in many cases leads to death within three to four years of diagnosis. It is also not possible to stop the disease from progressing.

The actual prognosis depends on many factors. Among other things, an early start of treatment has a decisive influence. But the already existing damage to the lungs and the speed at which the disease progresses also influence the success of the treatment. If further complications such as infections or cardiac insufficiency occur, the prognosis can deteriorate.

In order to positively influence the prognosis and avoid complications, it is necessary for the patient to adjust his lifestyle according to the advice of the attending physician.

Prevention

The only way to prevent pulmonary fibrosis is to prevent the triggering causes. Contact with toxic substances should be avoided and the employer must take protective measures, particularly in jobs where a worker comes into contact with such substances.

Aftercare

In the worst case, pulmonary fibrosis can lead to the death of those affected. If they undergo early treatment, the symptoms and limitations can often be treated well. Those affected primarily suffer from shortness of breath and shortness of breath, so physical exertion should be avoided as far as possible.

The lungs can no longer expand as usual and therefore cannot absorb enough oxygen. Those affected suffer from permanent tiredness and exhaustion and should rest as much as possible. The lower resilience can lead to various impairments in everyday life. The quality of life of those affected is usually significantly reduced by the disease.

Those affected are permanently dependent on the help and support of relatives. This can lead to depression and other mental illnesses. The condition can be treated with the help of various medications. Long-term medical care can have a lasting positive effect on the progression of pulmonary fibrosis.

You can do that yourself

Progressive pulmonary fibrosis is accompanied by increasing difficulty in breathing, because the lungs become less elastic as a result of the fibrosis and the chest muscles have to exert more force to expand the lungs for the process of inhalation. This, in turn, often causes those affected to breathe shallowly and intuitively take it easy on their bodies. As it turns out, such behavior is counterproductive.

The goal can be seen as an adjustment in everyday life and as a self-help measure, parallel to drug treatment aimed at stopping the fibrosis in the course of doing sports as far as possible. It is ideal if there is the opportunity to practice the sport in a lung sports group under medical supervision. The advantages of physical activity lie in an improvement in basic fitness and thus in an improvement in well-being. In addition, the chest muscles are trained and strengthened, so that breathing during the resting phase is apparently easier.

Another self-help measure consists of targeted breathing exercises, which aim to make better use of the existing capacity of the lungs and to train and strengthen the muscles necessary for breathing. Long-term oxygen therapy is now discouraged when pulmonary fibrosis has been diagnosed because there is a risk that the increased oxygen supply will promote so-called reactive oxygen species. This could accelerate the progression of pulmonary fibrosis.