In idiopathic pulmonary fibrosis (also idiopathic pulmonary fibrosis, IPF for short), connective tissue forms in the lungs in an uncontrolled manner. The result is severely restricted lung function. A cause of the disease is not known.
What is idiopathic pulmonary fibrosis?
According to abbreviationfinder, idiopathic pulmonary fibrosis has an insidious onset and is often initially symptomless. Only later do symptoms appear that resemble the symptoms of other forms of pulmonary fibrosis and those of heart failure.
Idiopathic pulmonary fibrosis is a chronic lung disease and a form of pulmonary fibrosis. The cause of the disease is not known. Therefore she is called “idiopathic” (from Greek idios – “self” and pathos – “suffering”). It is slowly progressing. As a result of excessive repair mechanisms, more and more connective tissue forms in the lungs as the disease progresses (fibrosing disease).
As a result, the lung tissue becomes increasingly stiff and loses elasticity. As a result, the lung function and especially the oxygen uptake into the blood are restricted. Idiopathic pulmonary fibrosis is rare, affecting men slightly more often than women. The diagnosis is usually made from the age of 60. Children are not affected. Compared to other forms of pulmonary fibrosis, idiopathic pulmonary fibrosis has a more rapid course. The therapies also differ.
The causes of idiopathic pulmonary fibrosis are not known. Older age and smoking are risk factors. Genetic factors, i.e. cases of idiopathic pulmonary fibrosis in the family, also seem to play a role. However, the complete genetic background has not yet been clarified. In the disease, a natural repair mechanism of the lungs runs uncontrolled:
Scar-like connective tissue forms as a reaction to small injuries in the area of the air sacs (alveoli). In idiopathic pulmonary fibrosis, this happens abnormally and an excessive amount of connective tissue forms. As a result, the lungs become less and less flexible and the lung function is increasingly restricted.
Symptoms, Ailments & Signs
Idiopathic pulmonary fibrosis has an insidious onset and is often initially symptomless. Only later do symptoms appear that resemble the symptoms of other forms of pulmonary fibrosis and those of heart failure. The patients suffer from shortness of breath.
This is particularly noticeable at first under load, but later also occurs at rest. In addition, the respiratory rate may be increased. Sufferers often have a persistent cough that responds poorly to expectorants. A quarter of the patients show drumstick fingers. The restricted lung function creates a lack of oxygen, which can lead to symptoms such as tiredness, blue discolouration of the lips and fingers, loss of appetite and weight loss.
Diagnosis & course of disease
The diagnosis of idiopathic pulmonary fibrosis can only be made if no cause for the disease can be identified. Therefore, possible causes of the symptoms are first ruled out in an anamnesis interview. Among other things, it is important whether the patient has had contact with potential triggers in their private or professional environment and whether they are taking medication that can trigger pulmonary fibrosis.
When listening to the lungs, the doctor may hear rales when inhaling. These sounds initially only occur in the lower lung sections, but as the disease progresses they can also occur in other areas of the lungs. Imaging methods are used for further diagnosis. X-rays and CT (computed tomography) of the chest show characteristic abnormal structures of the lung tissue and a reduced lung volume.
In the early stages of the disease, these changes are more visible on CT than on X-rays. If the CT findings are inconclusive, a lung biopsy may be necessary to confirm the diagnosis. Tissue samples are taken from the lungs, which are then evaluated under the microscope for diseased tissue (pathohistological).
Bronchial lavage can be used to rule out differential diagnoses. Here, the lungs are rinsed with a solution, which is then suctioned off and examined (cytologically) for certain cell occurrences. A pulmonary function test shows impaired lung capacity. Other forms of pulmonary fibrosis, drug-induced lung damage, COPD (chronic obstructive pulmonary disease), organizing pneumonia, diffuse alveolar damage and collagenosis (connective tissue disease) must be ruled out in the differential diagnosis.
Idiopathic pulmonary fibrosis begins insidiously. However, it then progresses faster than other forms of pulmonary fibrosis and has a poorer prognosis. On average, those affected live three years after diagnosis. Only 20 to 40 percent of those affected live five years or more after diagnosis. The lethality is 70 percent. However, the course of the disease can vary greatly from patient to patient.
The reasons for this are not known. Idiopathic pulmonary fibrosis can also promote other lung diseases and infections, which in turn can lead to acute worsening of pulmonary fibrosis. It then progresses even faster with a sudden massive deterioration in lung function and can be fatal.
Pulmonary fibrosis usually leads to a reduction in lung function. This can lead to various complaints in the patient and in most cases reduces the quality of life of the person concerned. The patient’s resilience is greatly reduced by the pulmonary fibrosis, which manifests itself in the form of exhaustion.
Those affected suffer from shortness of breath, which often leads to panic attacks or sweating. This can also result in heart problems, and in the worst case the patient can die of cardiac death. Fatigue and weight loss occur. Due to the lack of oxygen, internal organs and extremities can be damaged or die off completely.
There is also an increase in respiratory rate and a strong cough. Complications usually arise when the treatment of pulmonary fibrosis is started late. This is done with the help of medication and can limit the symptoms. In severe cases, a lung transplant is necessary to keep the patient alive. Under certain circumstances, life expectancy is limited and reduced by pulmonary fibrosis.
When should you go to the doctor?
Idiopathic pulmonary fibrosis does not initially cause any clear symptoms and symptoms. A doctor should be consulted if there is sudden shortness of breath or a persistent cough that cannot be attributed to a specific cause. At the latest when severe tiredness and external changes occur, medical advice is required. People who notice blue discoloration of their lips and fingers or weight loss should get this checked out immediately. If drumstick fingers occur, a medical professional should be consulted the same day.
This applies in particular when there are additional risk factors such as alcohol and nicotine consumption or old age. Even after long-term lung diseases, there is an increased risk of developing idiopathic pulmonary fibrosis. If complications such as persistent breathing problems or severe fatigue occur, a visit to the hospital is indicated. In the event of impaired consciousness and acute shortness of breath, the emergency doctor must be called. The treatment should be carried out by the family doctor or by a lung specialist. Depending on the cause, a cardiologist may also be consulted.
Treatment & Therapy
The therapy of idiopathic pulmonary fibrosis has no decisive influence on the survival and the quality of life of the affected person. While other forms of pulmonary fibrosis can be treated with corticosteroids and immunosuppressants, idiopathic pulmonary fibrosis does not respond to these drugs. The aim of treating the disease is to slow down or, if possible, stop the abnormal formation of connective tissue.
Furthermore, the patient’s complaints are treated as part of a symptomatic therapy. In advanced stages, long-term therapy with oxygen is necessary. In suitable cases, a lung transplant can be performed as a last resort. The patient’s lungs are completely replaced with donor lungs.
Outlook & Forecast
Idiopathic pulmonary fibrosis is characterized by rapid fibrosis progression. This means that it has the worst prognosis of all forms of pulmonary fibrosis. Unlike other forms of pulmonary fibrosis, the cause of the idiopathic variant is unknown. Therefore, no underlying disease can be treated to stop the progression of the disease. Various medications can slow down the progression of the disease, but there are still no long-term results that allow a reliable prognosis. It is not possible to cure idiopathic pulmonary fibrosis, at most the symptoms associated with treatment can be alleviated.
As a result of the connective tissue remodeling of the lungs, various complications can occur that significantly worsen the prognosis. These include changes in the heart that lead to right heart failure as a result of the increase in pressure in the pulmonary circulation over the course of the disease.
The lethality of idiopathic pulmonary fibrosis is around 70 percent. Patients with idiopathic pulmonary fibrosis live approximately three years after diagnosis. The 5-year survival rate is between 20 and 40 percent. This means that five years after the diagnosis was made, 20 to 40 out of 100 patients are still alive. However, the quality of life is significantly reduced, especially in advanced stages of the disease. For example, patients are often dependent on long-term therapy with oxygen or have to wait for a donor lung for organ transplantation.
Other forms of pulmonary fibrosis can be prevented by avoiding the triggers. Since no causes are known for idiopathic pulmonary fibrosis, this is not possible in this case. However, smoking is considered a risk factor that can be avoided.
The aftercare of idiopathic pulmonary fibrosis is about slowing down the progressive development of the connective tissue. This can only happen as part of medical treatment. Patients therefore need long-term medical care that includes regular check-ups.
While there are no specific suggestions for self-help, people can stop smoking to protect themselves. If other triggers for the occurring symptoms are known, these should also be avoided as much as possible. In the aftercare phase, drug treatment must be followed exactly as recommended by the doctor.
Furthermore, those affected should make sure that they do not overexert themselves physically. Difficulty breathing can indicate a lung infection, so it makes sense to see a doctor early. Depending on the severity of the illness, family members and friends should know so that they can help if necessary.
Loving care strengthens the patients and ensures an improvement in their state of health. If necessary, psychological support can also be helpful. Contact with other sufferers also has a positive effect on the patient’s mood and recovery.
You can do that yourself
As a rule, those affected by this disease have no options for self-help. For this reason, patients are always dependent on medical treatment.
However, smoking can be a risk factor for the disease, so the affected person should refrain from smoking. If another trigger is known that leads to the symptoms of the disease, this must also be avoided. Patients are dependent on taking medication. Physically strenuous activities should be avoided in everyday life. The risk of infections and inflammation of the lungs can also be increased, so a doctor should always be consulted immediately if you have difficulty breathing.
In severe cases, patients depend on the support of friends and family. Warm-hearted care has a positive effect on the course of the disease. Psychological support may be necessary for a lung transplant. This can also be done by relatives and friends, whereby contact with other patients can also have positive effects on the patient’s mental state.