Internal Medicine

The pulmonary fibrosis

What is pulmonary fibrosis?

With pulmonary fibrosis Due to various factors, there is an irreversible (irreversible) change in the lung tissue. Under fibrosis one understands the increase of connective tissue in an organ.

This is particularly problematic in the lungs because the lungs lose their natural elasticity due to the increase in connective tissue. More force needs to be applied to the chest breathing movements. The passage of oxygen from the alveoli into the blood vessels is also made more difficult by the increasing layer of connective tissue. Therefore, the oxygen concentration in the blood decreases.

The consequences are significantly reduced performance and even shortness of breath, even at rest. Pulmonary fibrosis is therefore a serious chronic disease.

Causes of pulmonary fibrosis

There are many causes of pulmonary fibrosis. A distinction is first made between known and unknown causes. Unfortunately, a cause for the pulmonary fibrosis cannot be found in a large number of patients. One then speaks of one idiopathic pulmonary fibrosis. In addition to unknown causes, there are also numerous researched triggers for pulmonary fibrosis.

This includes breathing in harmful substances such as asbestos or quartz dust. But also the cigarette smoke can cause pulmonary fibrosis. This also applies to certain drugs, e.g. Chemotherapy drugs or amiodarone, this is used to treat irregular heartbeat.

Must be the chest as part of cancer therapy Irradiation can also damage the lungs and lead to fibrosis.

For certain rheumatic diseases such as Rheumatoid arthritis or lupus erythematosus are also at risk of developing pulmonary fibrosis. This also applies to diseases such as amyloidosis or sarcoid. In sarcoid in particular, the lungs are often involved, which, if left untreated, ultimately leads to pulmonary fibrosis.

For more information on amyloidosis, see this topic: Storage diseases - what are there?

Are there any hereditary causes?

Yes, there are also hereditary causes of pulmonary fibrosis.
Especially with idiopathic pulmonary fibrosis (IPF) Genetic factors play a role. In idiopathic forms of pulmonary fibrosis, no clear trigger can be determined. However, we now know that there is a certain accumulation of this disease in families of those affected. It therefore stands to reason that genetic or hereditary factors also play a role. However, this has not yet been explored in detail.

It's just that the relatives of patients themselves have a Disposition have pulmonary fibrosis, i.e. are slightly more susceptible to the disease than the normal population. You don't necessarily have to develop pulmonary fibrosis, however. However, it is important that people with a genetic disposition to pulmonary fibrosis do not smoke under any circumstances. By consuming nicotine you have a significantly higher risk of the disease breaking out.

Also read: How to quit smoking

Pulmonary fibrosis after radiation

Radiation is sometimes necessary to treat tumor diseases.
In the case of breast, lung or lymph gland cancer, radiation in the chest area may be necessary. Even if the tumor and the corresponding lymphatic drainage can be irradiated very precisely today, the lung tissue is sometimes also affected. In the course of the proliferation of connective tissue, irreversible (= non-reversible) pulmonary fibrosis can develop.

Further information on this: Long-term effects after radiation treatment

Pulmonary fibrosis and rheumatism

Lung diseases in rheumatics are not uncommon. In addition, anti-rheumatic drugs can also damage the lungs.
In the event of complaints such as coughing or shortness of breath during exertion, you should immediately think of lung damage. Pulmonary fibrosis can occur in all diseases of the rheumatic type.

It mainly affects patients with scleroderma or lupus erythematosus. But a patient with rheumatoid arthritis, the typical rheumatoid arthritis, can also develop a lung disease. Smoking and treatment with special drugs are considered risk factors for developing pulmonary fibrosis (TNF inhibitors). This form of pulmonary fibrosis can be treated through rheumatism with cortisone or other immunosuppressants.

You might also be interested in this: Rheumatoid arthritis

Lung fibrosis after chemotherapy

As part of cancer treatment, patients often have to undergo chemotherapy in addition to radiation. Even if this may cure the patient of the cancer, it is possible that the chemotherapy may have long-term effects. This is best studied for Bleomycin.

The risk of developing pulmonary fibrosis during chemotherapy is particularly high with high doses, with combination therapies with other drugs that are harmful to the lungs, in old patients, with additional radiation in the chest area and reduced kidney function.

Also read: Chemotherapy side effects

I recognize pulmonary fibrosis by these symptoms

In the early stages, the symptoms of pulmonary fibrosis are often non-specific. With chronic cough and increasing shortness of breath during exercise, one should think of a lung disease.
Mostly it is a dry, tickly cough. However, a fever can also occur. Then sometimes the wrong diagnosis of pneumonia is made.
As with other advanced lung diseases, patients with end-stage pulmonary fibrosis suffer from shortness of breath even at rest.

More information is available here: Symptoms of pneumonia

Diagnosis of pulmonary fibrosis

Pulmonary fibrosis is a very diverse disease and is therefore often not easy to diagnose. However, the symptoms such as chronic cough and Difficulty breathing during exercise. In the anamnesis, you can then specifically determine whether there are triggers for pulmonary fibrosis. The physical exam is followed by a series of exams.

The first exam will certainly be a lung function check. This can even be done in a family doctor's office. Here the suspicion of pulmonary fibrosis is confirmed. Then the path continues with the specialist. This is where imaging studies are like a X-ray image and a Computed tomography carried out. High-resolution computed tomography is the most important diagnostic tool. An unremarkable finding in this examination rules out pulmonary fibrosis.

You might also be interested in: The pulmonary function test

X-ray of the lungs

In the case of pulmonary fibrosis, the conventional chest x-ray usually shows abnormalities. Radiologists refer to these as Drawing multiplication the structure of the lungs. Actually, it should air-filled lung tissue in the X-ray represent black, White appear Blood vessels and Connective tissue septa.

In the case of pulmonary fibrosis, there is increased connective tissue remodeling of the lung tissue. This increase in connective tissue can be seen in the X-ray. The examination of choice to confirm the diagnosis is high-resolution computed tomography (CT).

Read also:

X-ray of the chest
CT of the lungs

Treatment and therapy of pulmonary fibrosis

The connective tissue remodeling of the lung tissue in the context of pulmonary fibrosis is usually irreversible. There is no causal treatment for most forms of pulmonary fibrosis. Important therapeutic goals are therefore to prevent the further progression of the disease by eliminating possible triggers, and to improve the quality of life by treating the symptoms as effectively as possible.

If the causes of pulmonary fibrosis are known, they should be eliminated. A change of job must be initiated for patients who inhale dusts due to their work.
If the cause is a rheumatic disease, this should be treated as optimally as possible.
Pulmonary fibrosis itself often has an inflammatory component, which is why many patients are treated with cortisone.

However, patients with the idiopathic form of pulmonary fibrosis usually respond less well to treatment. There are now newer drugs that are approved for treating the idiopathic form such as Pirfenidone and Nintedanib. With these one tries to prevent a further progression of the disease.

The last option for young patients with end-stage pulmonary fibrosis is a lung transplant. For symptomatic therapy, all patients from a certain stage onwards receive long-term oxygen therapy, in which the patients receive supplementary oxygen almost the whole day.

Is pulmonary fibrosis curable?

Pulmonary fibrosis is not curable to the present day of science.
The connective tissue changes that have already taken place in the lungs are also irreversible. In addition, it depends on the cause of the pulmonary fibrosis whether the disease can be stopped in its progression. In the idiopathic forms, the trigger for pulmonary fibrosis is not known. There is therefore no causal therapy. The disease usually progresses. There is no cure at this point.

Science is of course working flat out to keep researching new drugs that can at least significantly slow down the course of the disease. But so far there has been no research breakthrough.
This is particularly stressful for young patients, as life expectancy is significantly reduced. The only hope for these patients is a lung transplant. With the transplant, the patients gain significantly more quality of life and also a little more life. 5 years after a lung transplant, 70% of the transplant recipients are still alive, and new immunosuppressive drugs have steadily improved this number. Due to the lack of donor organs and the severity of the operation, however, lung transplantation is only an option for a few people.

Further information on the subject is available here: The lung transplant

Stages of pulmonary fibrosis

In the early stages of pulmonary fibrosis, patients initially only suffer from a cough and shortness of breath when exerted.

Most of the time, however, the disease continues to develop. In the advanced stages, the symptoms are more severe. The lack of oxygen in the blood leads to cyanosis (Blue color of the lips). Even at rest, the patients have difficulty breathing. The load capacity is extremely limited. The increased pressure in the pulmonary circulation also puts stress on the right heart.

The end stage of pulmonary fibrosis is a "honeycomb lung", the lung tissue has receded so much that it has largely been replaced by connective tissue cavities.

You might also be interested in this topic: Pleural mesothelioma

Disease course of pulmonary fibrosis

Pulmonary fibrosis is not a uniform clinical picture. Rather, it is a collective term for many diseases in which there is an increase in connective tissue in the lungs. The course of the disease is therefore often very different. Mostly it depends on whether you can identify and eliminate the triggering factor. Even if in most cases there is no causal therapy and the connective tissue remodeling is irreversible, an early diagnosis is important to promote a lung-friendly lifestyle. Smoking accelerates the course of the disease immensely. Absolute nicotine abstinence is recommended for patients with pulmonary fibrosis.

You might also be interested in: Quit Smoking - But How?

The cause of the idiopathic form of pulmonary fibrosis is unknown. The disease is usually chronically progressive, which means it progresses over time. The symptoms worsen, and in the end stage patients suffer from shortness of breath even at rest. Long-term oxygen therapy is necessary. The increasing pressure in the pulmonary circulation increases the stress on the right half of the heart. The patients then suffer from right heart failure. In the end stage of pulmonary fibrosis, the patients are very seriously ill and can hardly get out of bed. Most end-stage patients die.

Life expectancy with pulmonary fibrosis

The prognosis of pulmonary fibrosis depends primarily on its cause. If the trigger is known and it could be switched off early, the prognosis is better. However, if it is an idiopathic form, the progression of which is difficult to prevent, the prognosis is poor.

Basically, pulmonary fibrosis is a serious disease that usually leads to a significant deterioration in quality of life. Life expectancy is also significantly limited. It is usually two to five years after diagnosis, as there is unfortunately still no causal therapy for most forms. Lung transplantation is therefore usually the only hope for younger patients with pulmonary fibrosis.

Read our article on this: Life expectancy with pulmonary fibrosis

This is what end-stage pulmonary fibrosis looks like

Patients with terminal pulmonary fibrosis are very seriously ill. Most of them die.
The patients are bedridden. You suffer from shortness of breath even at rest. The lips are discolored bluish (cyanosis). Physical strain is hardly possible any more. All patients are dependent on additional oxygen almost every minute. This is also possible at home as part of long-term oxygen therapy.
Due to the increased stress on the right heart, the patients also suffer from symptoms of a right heart failure with leg edema, neck vein congestion and ascites due to congestive liver. Lung transplants are no longer possible at such a stage, so most patients die in the process.

Is pulmonary fibrosis contagious?

No, pulmonary fibrosis is not caused by viruses or bacteria. Infection is therefore not possible.
However, pulmonary fibrosis can be developed if you inhale asbestos or dusty fumes like someone else does. These poisons damage everyone's lungs. However, contact with patients with pulmonary fibrosis is not contagious. Even with the idiopathic form, here the cause of the pulmonary fibrosis is unknown, one thinks more of genetic factors, an infectious disease is practically impossible.