In idiopathic pulmonary fibrosis (also called idiopathic pulmonary fibrosis, or IPF ), connective tissue forms uncontrollably in the lungs. The result is severely impaired lung function. There is no known cause of the disease.
What is idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis is a chronic lung disease and a form of pulmonary fibrosis. The cause of the disease is not known. Therefore, it is called “idiopathic” (from Greek idios – “self” and pathos – “suffering”). It is slowly progressive. 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, lung function and especially oxygen uptake into the blood are restricted. Idiopathic pulmonary fibrosis is rare and affects men slightly more often than women. It is usually diagnosed after the age of 60. Children are not affected. Compared with other forms of pulmonary fibrosis, idiopathic pulmonary fibrosis has a more rapid course. Therapies also differ.
Causes
The causes of idiopathic pulmonary fibrosis are unknown. Risk factors include older age and smoking. Genetic factors, that is, cases of idiopathic pulmonary fibrosis in the relatives, also appear to play a role. However, the full genetic background is not yet clear. In the disease, a natural repair mechanism of the lung runs uncontrolled:
Scar-like connective tissue forms in response to small injuries in the area of the alveoli (air sacs). In idiopathic pulmonary fibrosis, this occurs pathologically and an excessive amount of connective tissue is formed. As a result, the lungs become less and less expandable and lung function becomes more and more limited.
Symptoms, complaints, and signs
Idiopathic pulmonary fibrosis has an insidious onset and often progresses without symptoms at first. Only as it progresses do symptoms appear that resemble those of other forms of pulmonary fibrosis and heart failure, among others. Patients suffer from shortness of breath. This is initially particularly noticeable during exertion, but later also occurs at rest. In addition, the respiratory rate may be increased. Patients often suffer from a persistent cough that responds poorly to cough suppressants. One quarter of patients present with drumstick fingers. Impaired lung function results in a lack of oxygen, which can lead to symptoms such as fatigue, a blue tinge to the lips and fingers, loss of appetite, and weight loss.
Diagnosis and disease progression
The diagnosis of idiopathic pulmonary fibrosis can only be made if no cause for the disease is identified. Therefore, possible causes of the symptoms are first ruled out in a medical history interview. Among other things, it is important to know whether the patient has had contact with potential triggers in his or her private or professional life and whether the patient is taking medication that can trigger pulmonary fibrosis. When listening to the lungs, the physician may hear rales when breathing in. These sounds occur initially only in the lower portions of the lungs, but as the disease progresses, they also occur in other areas of the lungs. Imaging techniques are used in further diagnosis. X-rays and CT (computed tomography) scans of the chest show characteristic abnormal structures of the lung tissue and decreased lung volume. In early stages of the disease, these changes are more readily seen on CT than on x-ray. If the CT findings are inconclusive, a lung biopsy may be necessary to confirm the diagnosis. This involves taking tissue samples from the lungs, which are then evaluated under the microscope for diseased tissue (pathohistologically). A bronchial lavage can be used to exclude differential diagnoses. This involves flushing the lungs with a solution, which is then aspirated and examined for specific cell occurrences (cytologically). A pulmonary function test reveals impaired lung performance. Differential diagnoses should exclude other forms of pulmonary fibrosis, drug-induced lung damage, COPD (chronic obstructive pulmonary disease), organizing pneumonia, diffuse alveolar damage, and collagenosis (connective tissue disease).Idiopathic pulmonary fibrosis begins insidiously. However, it then progresses more rapidly than other forms of pulmonary fibrosis and has a poorer prognosis. On average, those affected live for three years after diagnosis. Only 20 to 40 percent of sufferers live for five years or longer after diagnosis. The lethality rate 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 favor other lung diseases and infections, which in turn can lead to acute worsening of pulmonary fibrosis. It then progresses even more rapidly with sudden massive deterioration of lung function and can be fatal.
Complications
Pulmonary fibrosis usually results in reduced lung function. This can lead to various symptoms in the patient and, in most cases, reduces the quality of life of the affected person. The patient’s ability to cope with stress is greatly reduced by pulmonary fibrosis, which becomes apparent in the form of fatigue. Patients suffer from shortness of breath, which often leads to panic attacks or sweating. Heart problems may also result, and in the worst case the patient may die of cardiac death. Fatigue and weight loss occur. Due to the undersupply of oxygen, internal organs and extremities can be damaged or die completely. Furthermore, there is an increase in respiratory rate and a severe cough. As a rule, complications occur when treatment of pulmonary fibrosis begins late. This is done with the help of medication and can limit the symptoms. In severe cases, transplantation of a lung is necessary to keep the patient alive. In some circumstances, life expectancy is limited and reduced by pulmonary fibrosis.
When should you see a doctor?
Idiopathic pulmonary fibrosis does not initially cause definite symptoms and symptoms. A physician should be consulted if there is sudden shortness of breath or a persistent cough that cannot be attributed to any specific cause. Medical advice is required at the latest when severe fatigue and external changes are added. Individuals who notice a blue discoloration of the lips and fingers or weight loss should have this clarified immediately. If drumstick fingers occur, a medical professional should be consulted the same day. This is especially true if risk factors such as alcohol and nicotine use or older age are added. There is also an increased risk of developing idiopathic pulmonary fibrosis after prolonged lung disease. If complications such as persistent breathing problems or severe fatigue occur, a visit to the hospital is indicated. In case of impaired consciousness and acute respiratory distress, the emergency physician must be called. Treatment should be provided by the primary care physician or by a pulmonary specialist. Depending on the cause, a cardiologist may also be consulted.
Treatment and therapy
Treatment of idiopathic pulmonary fibrosis does not have a decisive impact on the survival and 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 medications. Therapy of the disease aims to slow down or, if possible, stop the pathological formation of connective tissue. Furthermore, symptomatic therapy is used to treat the patient’s symptoms. In advanced stages, long-term therapy with oxygen is necessary. In suitable cases, lung transplantation can be performed as a last resort. In this procedure, the diseased person’s lung is completely replaced by a donor lung.
Outlook and prognosis
Idiopathic pulmonary fibrosis is characterized by rapid progression of fibrosis. Thus, it has the poorest prognosis among all forms of pulmonary fibrosis. Unlike other forms of pulmonary fibrosis, the cause of the idiopathic variant is unknown. Thus, no underlying disease can be treated to halt the progression of the disease. Various medications can slow 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 best, 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 during the course of the disease. The lethality of idiopathic pulmonary fibrosis is around 70 percent. After diagnosis, patients with idiopathic pulmonary fibrosis live for approximately three years. The 5-year survival rate is between 20 and 40 percent. This means that five years after diagnosis, 20 to 40 out of 100 patients are still alive. However, the quality of life is significantly limited, especially in advanced stages of the disease. For example, patients are often dependent on long-term oxygen therapy or must wait for a donor lung for organ transplantation.
Prevention
Other forms of pulmonary fibrosis can be prevented by avoiding their respective triggers. Because there are no known causes in idiopathic pulmonary fibrosis, this is not possible in this case. However, smoking is considered a risk factor that can be avoided.
Follow-up
Follow-up care of idiopathic pulmonary fibrosis is about slowing down the progressive further development of the connective tissue. This can only be done in the context of medical treatment. Patients therefore require long-term medical care that includes regular check-ups. Although there are no specific suggestions for self-help, those affected can stop smoking to protect their organism. If other triggers for the symptoms that occur are known, these should also be avoided as much as possible. Medicinal treatment should be followed exactly according to the doctor’s recommendations during the aftercare phase. Furthermore, those affected should take care not to exert themselves too much physically. Breathing difficulties may indicate an infection of the lungs, which is why an early visit to the doctor is advisable. Depending on the severity of the disease, family members and friends should know about it so that they can help if necessary. Loving care strengthens the patient and ensures an improvement in health. If necessary, psychological support is also helpful. Contact with other sufferers also has a positive effect on the patient’s mood and recovery.
What you can do yourself
As a rule, there are no self-help options available to the affected person with this disease. For this reason, patients are always dependent on medical treatment. However, smoking may be a risk factor for the disease, so the affected person should refrain from smoking. If another trigger is known, which leads to the symptoms of the disease, it must also be avoided. Patients are dependent on taking medication. Physically strenuous activities must be avoided in their daily lives. The risk of infections and inflammations of the lungs may also be increased, so that a doctor should always be consulted immediately if breathing problems occur. In severe cases, patients rely on the support of friends and family. In this case, warm-hearted care has a positive effect on the course of the disease. In the case of a lung transplant, psychological support may be necessary. This can likewise be provided by family members and by friends, and contact with other patients can also have a positive effect on the patient’s psychological state.
