Pulmonary fibrosis is a disease of the lungs that can have many different causes. It results in stiffening of the lungs due to scarring. Breathing difficulties and decreased exercise capacity are typical. Pulmonary fibrosis cannot be cured, only alleviated.
What is pulmonary fibrosis?
Infographic on the different lung diseases and their characteristics, anatomy, and location. Click to enlarge. Pulmonary fibrosis is a chronic inflammation of the lungs that usually starts with inflammation of the alveoli (air sacs). This inflammation leads to increased formation of connective tissue in the lungs, and the lung tissue becomes scarred and stiff. As a result, the expandability and volume of the lungs decrease sharply, and gas exchange in the lungs is severely impaired. Not only is breathing itself more difficult, but the scarring also makes it more difficult for oxygen itself to reach the blood, and shortness of breath is increased. The longer the pulmonary fibrosis persists, the heavier the burden on the heart. If there is no cause for the pulmonary fibrosis, the doctor refers to it as idiopathic pulmonary fibrosis.
Causes
Pulmonary fibrosis can have a variety of causes, but it always involves the uncontrolled formation of the body’s own collagen. Collagen is almost inextensible and, as the most important protein, is found everywhere in the human body. It is one of the most important components of human connective tissue and is thus found primarily in bones, teeth, cartilage, tendons, ligaments and, of course, the skin. 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 the past years was asbestos, which was used everywhere in the construction industry. Inhalation of asbestos fibers over a long period of time has been shown to cause inflammatory reactions and the development of pulmonary fibrosis. However, in half of the cases, the cause is also unknown.
Symptoms, complaints, and signs
Fibrosis of the lung may go undetected for years. The first symptoms do not appear until the organ is already severely damaged. Signs of the disease include shortness of breath and irritable cough, although breathing difficulties initially occur only during physical exertion. Only in the advanced stages do sufferers also experience shortness of breath at rest. Accompanying this, a pressing feeling of tightness may be noticed when breathing in. In individual cases, breathing is blocked and the lungs only open again after a few breaths. The breathing difficulties eventually lead to an oxygen deficiency in the body. This can be recognized by the blue-stained fingers and the so-called drumstick fingers, which are manifested by distended finger end links. The signs of the disease also include conspicuously curved fingernails and pallor. Individuals who have pulmonary fibrosis suffer excessively from respiratory and lung infections. In the worst case, pneumonia can occur, 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, fatigue, fever, loss of appetite and weight loss. In advanced stages, pulmonary hypertension often develops, which can lead to heart damage and water retention in the abdomen.
Diagnosis and progression
Patients with pulmonary fibrosis often suffer from cough, fatigue, and severely reduced exercise tolerance. An initial symptom is also water retention in the lungs (pulmonary edema), as the walls of the alveoli thicken. Gas exchange between blood and lungs is impeded. Initially, there is shortness of breath under exertion; in advanced stages, shortness of breath also occurs at rest. The respiratory rate is increased because the patient has to breathe in against a resistance. If 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 become bulbous, thickened and rounder than normal fingernails or toenails (drumstick fingers), and bulge like a watch glass (watch glass nails). In general, these are signs of prolonged tissue hypoxia. An initial indication of pulmonary fibrosis can be provided by auscultatory findings (i.e., the stethoscope).In addition to questioning the patient, the examination includes lung function tests and X-rays. The diagnosis is confirmed by a bronchoscopy, during which the physician takes samples of the lung tissue.
Complications
In the worst cases, pulmonary fibrosis can lead to the patient’s death. However, with early treatment, the limitations and symptoms can be treated and resolved relatively well. In most cases, those affected suffer from shortness of breath in the process and continue to suffer from shortness of breath. The lungs can no longer expand in the usual way and thus can no longer absorb sufficient oxygen. As a result, those affected feel fatigued and tired. There is also a reduced ability to work under pressure, so that patients no longer take an active part in their lives. Furthermore, a severe cough also occurs, which can lead to various restrictions in the patient’s daily life. In general, the quality of life of the affected person is significantly limited and reduced by pulmonary fibrosis. Pulmonary fibrosis can be treated with the help of various medications. In this case, no further complications occur. However, this treatment does not lead to a positive course of the disease in every case. It is therefore not uncommon for those affected to be dependent on a lung transplant in order to continue to survive. In many cases, only slowing the progression of pulmonary fibrosis is also possible.
When should you see a doctor?
If shortness of breath or difficulty breathing occurs, a doctor should be consulted. A visit to the doctor is necessary if the cough persists or if there is an irritating cough. The disease usually goes unnoticed initially because the patient is in a symptom-free state for a long time. This leads to the fact that the symptoms only appear in an advanced stage of the disease, which should then be classified as soon as possible. If the affected person suffers from anxiety due to the restricted breathing activity, a doctor should be consulted. If breathing blocks or if there are irregularities during inhalation, a doctor is needed. If there are signs such as fatigue, faintness, lassitude, or rapid fatigue, a physician should be consulted. If the usual performance can no longer be achieved, a comprehensive examination is advisable. If there is pain in the joints, bones or muscles, there is cause for concern. A decrease in body weight, the formation of swellings or discrepancies in heart rhythm should be checked and clarified by a specialist. If water retention develops, there are abnormalities in the appearance of the skin or apathy occurs, a visit to the doctor is necessary. Discoloration of the fingers, a pallor of the skin or disturbances of the blood circulation in the limbs are signs that should be discussed with a doctor.
Treatment and therapy
Pulmonary fibrosis cannot be cured at the current state of medical research. Scarred tissue does not regress. Therefore, the main goal of therapy is to stop or at least slow the expansion of scarring and stiffening of the lungs. If a toxin is the cause of fibrosis, the patient must, of course, strictly avoid the triggering pollutant immediately. In the case of inflammatory processes, the doctor often prescribes cortisone preparations and immunosuppressants, i.e. drugs that inhibit the immune system and positively influence 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, there is an indication for lung transplantation. It is difficult to predict the success of therapy because there are many different triggers of pulmonary fibrosis and the disease can thus progress very differently.
Outlook and prognosis
Once pulmonary fibrosis has been diagnosed, no specific statements can be made about a general prognosis. The reason for this is that the course of the disease is not uniform and the clinical picture can be very complex. In certain cases, pulmonary fibrosis may have little or no effect on the patient’s life expectancy. In other cases, however, the disease may progress rapidly. If it is pulmonary fibrosis that occurs without any apparent cause, the prognosis is generally poor. In general, it can be said that pulmonary fibrosis is not curable.This means that the lung tissue, which has been altered by scarring, does not recover. Pulmonary fibrosis is classified as an extremely severe disease and in many cases leads to the death of the patient within three to four years of diagnosis. It is also not possible to stop the disease in its progress. The actual prognosis depends on many factors. Among other things, an early start of treatment has a decisive influence. However, existing damage to the lungs and the speed at which the disease progresses also influence the success of treatment. If further complications occur, such as infections or cardiac insufficiency, the prognosis may deteriorate. To positively influence the prognosis and avoid complications, it is necessary for the patient to adjust his or her lifestyle according to the advice of the treating physician.
Prevention
The only means of prevention against pulmonary fibrosis is to prevent the precipitating causes. Contact with toxic substances must be avoided, and especially in occupations where a worker comes into contact with such substances, it is essential that the employer pay attention to protective measures.
Aftercare
In the worst cases, pulmonary fibrosis can lead to the death of affected individuals. If they undergo early treatment, the symptoms and limitations can often be well managed. Affected individuals primarily suffer from shortness of breath and shortness of breath, so physical exertion should be avoided as much as possible. The lungs can no longer expand as usual and therefore cannot absorb sufficient oxygen. Those affected suffer from permanent fatigue and exhaustion and should take it easy as far as possible. The reduced resilience can lead to various impairments in everyday life. The quality of life of those affected is usually considerably restricted by the disease. Affected persons are permanently dependent on the help and support of relatives. This can lead to depression and other mental illnesses. The disease can be treated with the help of various medications. Permanent medical care can have a lasting positive effect on the progression of pulmonary fibrosis.
What you can do yourself
Progressive pulmonary fibrosis is accompanied by increasingly difficult inhalation because the lungs as a whole become less elastic due to the fibrosis, and to expand the lungs for the process of inhalation, the chest muscles must exert more force. This, in turn, often causes sufferers to breathe shallowly and intuitively take it easy on themselves physically. As it turns out, such behavior is counterproductive. As an adaptation in everyday life and as a self-help measure, the goal can be seen to be to exercise within the limits of one’s possibilities, parallel to a drug treatment aimed at stopping the fibrosis in its course. It is ideal if there is the possibility of exercising in a lung sports group under medical supervision. The benefits of sporting activity are an improvement in basic fitness and thus an improvement in well-being. In addition, the chest muscles are trained and strengthened so that breathing seems to be easier during the resting phase. Another self-help measure consists of targeted respiratory gymnastics, which aims 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 not recommended in cases where 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.
