Idiopathic interstitial pneumonia combines a large group of lung diseases that share the components of inflammation and lung scarring to varying degrees. The causes are unknown. Therapeutically, the inflammatory process is primarily suppressed so that the disease process is ideally stopped. If treatment fails, lung transplantation should be considered early on.
What is idiopathic interstitial pneumonia?
The main symptoms of idiopathic interstitial pneumonia are shortness of breath and a dry cough. At the beginning, the shortness of breath is only noticed during physical exertion and progresses as the disease progresses, so that shortness of breath at rest also occurs.
According to abbreviationfinder, idiopathic interstitial pneumonia is an umbrella term for a large group of diseases of the lungs characterized by an inflammatory response and pulmonary scarring (fibrosis) of the connective tissue, capillaries and/or alveoli. Various forms of the disease are subsumed under it, which differ in the extent of lung damage, pathology, symptoms, course of the disease and therapeutic options.
In 2002, the American Thoracic Society and the European Respiratory Society published a joint classification of idiopathic interstitial pneumonia. A total of seven forms of the disease are currently distinguished:
- idiopathic pulmonary fibrosis
- non-specific interstitial pneumonia
- cryptogenic organizing pneumonia
- acute interstitial pneumonia
- respiratory bronchiolitis with interstitial lung disease
- desquamative interstitial pneumonia
- lymphoid interstitial pneumonia.
The exact cause of idiopathic interstitial pneumonia is not known. Inflammation and fibrosis are the main pathogenetic factors. Based on lung biopsy results, the following concept for the development of the disease currently exists. Initially, there is damage to the air sacs (alveoli) in the lungs, which leads to activation of inflammatory cells.
These inflammatory cells release certain messenger substances that lead to the migration of white blood cells (leukocytes) into the alveoli. This in turn activates granulocytes, which now release proteases and toxic oxygen metabolites. This leads to further damage to alveoli and disruption of surfactant formation in the alveoli, which then collapse.
In addition, fibroblasts proliferate via an inflammation characterized by lymphocytes, which remodel the connective tissue, the capillaries and the alveoli to form scars. As a result, the alveoli are additionally cut off from the blood and oxygen supply and the further formation of scar areas increases. The remaining alveoli that are still ventilated are overinflated to compensate and form cystic cavities resembling honeycombs.
Symptoms, Ailments & Signs
The main symptoms of idiopathic interstitial pneumonia are shortness of breath and a dry cough. At the beginning, the shortness of breath is only noticed during physical exertion and progresses as the disease progresses, so that shortness of breath at rest also occurs. In the final stage, respiratory failure with respiratory exhaustion can occur.
The symptoms of respiratory insufficiency, shortness of breath and a dry cough are chronic in most patients, i.e. for months or years before the diagnosis is made. Due to the connective tissue remodeling, the gas exchange in the lungs is disrupted, resulting in a lack of oxygen (hypoxemia) in the blood. Hypoxemia causes the skin and mucous membranes to visibly turn bluish, a condition known as cyanosis.
In particular, the lips, fingers, and toes become cyanotic. Persistent cyanosis typically develops drumstick fingers and watch-glass nails. The disease usually manifests itself in the second to fourth decade of life. Patients with idiopathic pulmonary fibrosis are mostly older than 60 years.
Diagnosis & course of disease
The diagnosis of idiopathic interstitial pneumonia is made clinically, radiologically and pathologically. The first step is the anamnesis and physical examination. In terms of medical history, the current complaints, previous illnesses, medication intake and family history are queried.
During the physical examination, the lungs are percussed and auscultated with a stethoscope. A dry end-inspiratory crackle and fine-bubble rales can often be heard. As the disease progresses, cyanosis, watch-glass nails, and drumstick fingers may appear as skin manifestations. If idiopathic interstitial pneumonia is suspected, lung function tests and imaging diagnostics with an X-ray or high-resolution computed tomography (CT) are performed.
Typical signs are patchy, reticular compressions, bronchial bulges and honeycomb formation. If the diagnosis is still unclear after imaging, bronchoscopy with bronchoalveolar lavage and biopsy is indicated. Lung biopsy is generally the most reliable method for confirming the diagnosis of the disease and initiating appropriate therapy.
The course of the disease is sometimes very variable, but most diseases progress insidiously. In some cases, patients who have had a chronic cough or shortness of breath for months or years do not consult a doctor until late. As a result, the corresponding diagnosis is made late and fibrotic remodeling has already occurred.
This disease causes significant problems and complications in the lungs and airways. In severe cases, no therapy or treatment is possible and the affected person is dependent on a transplant of a donor lung. Those affected usually suffer from shortness of breath and a strong cough. Coughing up blood can also occur, which often triggers a panic attack.
Due to the breathing difficulties, organs and extremities are often not supplied with enough oxygen and can be damaged as a result. Likewise, the patient is no longer so strong and can not do any particular physical activities or sports. This disease severely limits the patient’s quality of life. The shortness of breath can also lead to loss of consciousness, in which the person concerned can injure himself by falling.
The disease is usually treated with medication. Unfortunately, no causal treatment is possible, so that the symptoms must be limited in particular. However, this is not always successful. If the treatment is unsuccessful and no lungs can be found for the person concerned, the patient’s death is almost inevitable.
When should you go to the doctor?
In the case of respiratory disorders, a doctor’s visit is required. If the abnormalities persist for several days or weeks, a doctor must examine the lung activity and the airways. Coughing, difficult breathing, pauses in breathing or a dry mouth are indications that should be followed up. If there is a feeling of pressure in the chest, if you tire quickly during physical activity and if you have breathing noises, you should see a doctor. The person affected often suffers from a gradual process of change. Since an organ transplant is necessary in severe cases, a timely check-up visit to a doctor at the first signs is advisable.
If the skin becomes discolored, the disease is already advanced. Blue discoloration of the lips, toes or fingers should therefore be presented to a doctor as soon as possible. If the person concerned has a vague feeling of illness, if he notices a general feeling of being unwell or if his usual performance level drops, a doctor should be consulted. Changes or deformations of the fingers are considered unusual and should be checked out by a doctor. If you are afraid, panicking or feeling suffocated, you should consult a doctor. If dizziness sets in, gait insecurity occurs or you can no longer carry out your usual sporting activities, you need to see a doctor to determine the cause. A doctor should be consulted in the event of sleep disturbances, increased irritability or disturbances in alertness.
Treatment & Therapy
The main goal of treatment is to prevent the progression of pulmonary fibrosis because it is irreversible. Known triggers should be eliminated and the acute or chronic inflammatory process aggressively suppressed. Two groups of drugs are mainly used in therapy.
On the one hand the glucocorticoids and on the other hand the immunosuppressants. The effectiveness of the medication varies depending on the type of disease. Idiopathic pulmonary fibrosis, for example, could not previously be treated effectively with glucocorticoids or immunosuppressants, but the preparations pirfenidone and nintedanib have now been approved.
There are antifibrotic drugs that slow down the progression of the disease. Other subtypes of idiopathic interstitial pneumonia respond well to anti-inflammatory therapy. Oxygen therapy should be considered in patients with hypoxemia at rest or during exercise. In many patients, the disease progresses chronically and irreversibly, despite therapy, so that a lung transplant should be considered at an early stage.
Outlook & Forecast
The prognosis of idiopathic interstitial pneumonia is individual and must be based on the patient’s personal state of health. Basically, it is classified as unfavorable, since a cure is not to be expected. According to the current scientific and medical status, the damage caused by the inflammatory process in the lungs cannot be repaired, despite all efforts.
In severe cases, there is a risk of organ transplantation or the premature death of the person concerned, as there is a risk of organ failure. If treatment is successful, the prognosis improves. If the diagnosis is made early and treatment is started as quickly as possible, the progression of the disease can be greatly influenced. The chronically progressing inflammation can be inhibited by the administration of medication.
If the route of a donor lung is necessary to maintain the patient’s survival, the usual risks and side effects of the surgical intervention must be considered. Nevertheless, there is the possibility of a significant improvement in health and a prolongation of life. However, if the organism rejects the donor organ, the prognosis worsens.
The disease triggers sequelae in many patients. Anxiety, lifestyle restrictions and the restructuring of everyday life can lead to stress and trigger new diseases. This leads to a worsening of the overall prognosis, as the psyche plays a significant part in the recovery process.
Since the exact causes of the disease complex are unknown, no specific preventive measures can be named at the moment. Only general behavior can be formulated. Nicotine, drug or medication abuse should be avoided. Physical activity and a healthy diet are generally beneficial.
In idiopathic interstitial pneumonia, follow-up care is closely related to therapy. The goal is to suppress the process of inflammation and prevent pulmonary fibrosis from progressing. In this way, ideally, the course of the disease can be halted. Doctors prescribe drugs that eliminate the triggers.
At the same time, the drugs actively counteract the inflammatory process. Patients should follow the doctor’s instructions exactly and take the antifibrotic agents as directed. In this way, they succeed in slowing down the further course of the disease. Depending on the type of condition, anti-inflammatory treatment may also be used.
If it is hypoxemia, the doctor may recommend oxygen therapy. When deciding on the right form of treatment, patients need specialist advice. It is also important to have regular check-ups to see how the disease is developing. Even with targeted therapy, the disease can progress irreversibly, and then a lung transplant may make sense.
The actual triggers for the disease are often not recognizable. That’s why there are no concrete aid measures, only assistance for a health-conscious lifestyle. Avoiding nicotine, drugs and medication together with a balanced diet and physical activity has a positive effect.
You can do that yourself
The number one concern for patients with Idiopathic Interstitial Pneumonia is to stop the inflammation in the lungs and to increase medical effortssupport. Due to the seriousness of the illness, the doctor’s instructions take precedence over all other information and self-help measures. In addition, all independent approaches must be discussed with the doctor beforehand.
Usually, patients with idiopathic interstitial pneumonia receive various medications that must be taken on time and regularly. Since these are immunosuppressive drugs, among other things, those affected are increasingly careful their immune system. not tostrongto charge. In this way, those affected apply appropriate hygiene measures and also avoid contracting infections when they come into contact with other people. In this sense, it is also beneficial to take regular rest in idiopathic interstitial pneumonia.
Sporting activities can often no longer be carried out to the same extent as before the illness. However, doing without physical training altogether is not necessary or beneficial in every case. The doctor often refers the patient to a physiotherapist, who sets up a training concept adapted to idiopathic interstitial pneumonia. As with all lung diseases, it is imperative to stop smoking immediately with idiopathic interstitial pneumonia.