What is IPF?

What is IPF?

• The full meaning of IPF disease is Idiopathic Pulmonary Fibrosis. It is a disease that progresses with tissue thickening in the lungs of unknown cause.

Why does it happen?

Although the cause is unknown, the following factors are held responsible:

• Repeated micro damage in different parts of the lungs,

• Continuation of the repair process,

• Wound healing being damaged,

• The inability of the alveolerepithelial cell layer to renew itself,

• Uncontrolled scar tissue formation, a wound in the body, damage to the body as a result of damage. Uncontrolled formation of scars and tissue groups remaining as a result of self-repair

• Viral infections, smoking, gastric reflux, exposure to wood or metal dust in the work environment,

• Insufficient renewal of the airway surface, whose integrity has been damaged due to aging

What is the frequency of occurrence?

• There is no clear data on this issue for Turkey. However, roughly 6-8% of the cases encountered in patients who apply are diagnosed with IPF.

• In the United States, it is 6.8-8.8 per 100,000 people in narrow cases and 6.8-8.8 per 100,000 people in broad cases. In cases, it is seen at a rate of 16.3-17.4 per 100,000 people. In Europe, this rate is between 0.22 and 74 per 100,000 people.

• Its prevalence increases by approximately 5 percent every year

What are the symptoms of the disease?

• Increasing shortness of breath,

• Persistent cough,

• Clubing in the fingers is the three most important findings.

In such cases, it is necessary to take a detailed and careful history from the patient. The information received from patients becomes a very important guide. In most patients, clinical findings began long before consulting a doctor. During this period, patients may not be aware of the limitation of movement at first, as they adjust their activities according to their current performance.

Which is the most striking and important among all the symptoms?

• Over the age of fifty Patients who have smoked and have the complaints mentioned above should be evaluated for IPF.

What are the factors that trigger the emergence of the problem?

• Smoking, environmental factors, some virus infections (EBV; HCV, CMV, HHV-7, HHV-8), gastric reflux, some familial factors (familial cases are younger), and gene disorders.

Who is at risk in this disease?

• People who form the infrastructure mentioned above can be considered at risk.

Is the disease more common in women or men?

Is the disease more common in women or men? p>

• It is more common in men, but it is seen to a considerable extent in women.

• According to an epidemiology study conducted in New Mexico, the prevalence is 81 / 100 000 in men. It is reported that it is 67 / 100 000 in women and 32 / 100 000 new patients are added every year in men and 26 / 100 000 new patients in women.

Are there any diseases with which it is confused?

• The items that we can list at once are as follows: Differential diagnosis of patients in this group, which can be confused with infectious causes, from community-acquired pneumonia and fungal infections caused by viruses, mycoplasma bacterial species or legionella bacterial species, and acute respiratory distress syndrome.

• Lung diseases that develop due to external organic matter intake, such as sarcoidosis, lung thickening due to connective tissue disease, genetic lung diseases such as alveolar microlithiasis, Gaucher disease, and eosinophilic granulomas, are mostly in the young age group ( 20-40 years old) is seen. Lung fibrosis due to genetic diseases such as lymphangiomyomatosis and tuberous sclerosis is more common in women than in men. In men, lung thickening occurs mostly due to occupational and environmental factors such as pneumoconiosis, silicosis, asbestosis and berylliosis. It is necessary to take a detailed retrospective occupational and local history. Since the latent period of some lung thickenings is very long, former occupations and forgotten exposures should be questioned one by one.

Is it difficult to diagnose the disease? If your answer is "yes", why?

• It is essential to make a differential diagnosis and perform a detailed examination. Opposite Otherwise, it is not possible to choose the appropriate treatment and exclude other diseases. A detailed disease history, physical examination, laboratory examinations, filmography and, if necessary, bronchoscopy are used to make a differential diagnosis and exclude other diseases.

How is the diagnosis made?

• Thin rales (Velcro rales) that completely cover inspiration (that is, during breathing) are important in diagnosis. It is not possible for the patient to understand this. It is a finding that the physician will detect during the examination.

• The most important component of the diagnostic phase in IPF is high-resolution computed tomography (lung film taken with a special method)

• If the diagnosis refers to ordinary interstitial pneumonia, tissue diagnosis is usually not required. However, if a decision cannot be made with CT, it is useful to take a lung biopsy. Generally, Flexible bronchoscopy (the process of entering the lung through the mouth with a lighted optical device) transbronchial biopsy (usually taken with fluoroscopy and imaging) together with bronchoalveolar lavage (lung wash water), if a decision cannot be made, cryobiopsy with Flexible bronchoscopy and if still not decided, the surgical method called open lung biopsy is applied. . There are very few cases that are referred to surgery. In older patients, the risk of surgery should also be carefully calculated. For the diagnosis, the experienced bronchoscopist, clinician, pathologist and radiologist must work in harmony, and the biopsy taken must show compliance with the diagnosis, that is, ordinary interstitial pneumonia.

Flexible bronchoscopy is the examination of the lungs through the mouth with an optical instrument. process. It is applied with simple sedoanalgesia or a short induction anesthesia. Biopsy is taken with this method.

Is there a treatment? If your answer is yes, what do we need to understand about treatment?

There are guidelines that guide physicians at certain year intervals, including diagnosis and treatment options in the light of scientific foundations, in the approach to diseases affecting the lung tissue. Physicians diagnose their patients and make treatment plans according to these guidelines. According to the American Respiratory Society (ATS) and European Respiratory Society (ERS) guidelines revised in April 2015, the recommended active ingredient in this disease is pirfenidone. Another recommended active ingredient is Nintadenib, a tyrosine kinase inhibitor. is. Pirfenidone received approval from the US Drug Administration in 2014. Cortisone therapy, immunosuppressants, and the expectorant drug called n-acetylcysteine, which are used in attacks from time to time, are now less included in the treatment.

• Pirfenidone, although its exact mode of action is not known. It is a pyridone compound that has been shown to have antifibrotic, anti-inflammatory and antioxidant properties in animal models.

• Another molecule that was later approved is Nintedanib. This tyrosine kinase inhibitor is a drug and is available and used in our country

• It has been concluded that the increase in forced vital capacity slows down the rate of progression of the disease

• Following this study, CAPACITY and ASCEND studies were conducted and with the positive results found, it was decided to include this drug in the guidelines.

• In cases with rapid progress with treatment, if there is no contrary condition, if they are before the age of 65 and if they are before the age of 65 and they are given no other supplements. If there is no disease, lung transplantation is recommended.

 

What does early diagnosis provide to the patient?

The average survival after diagnosis is 2-3 years. However, this is not the rule; although there are forms that progress very quickly, there are also cases that have a 10-year course and do not flare up at all. Early diagnosis reduces the areas lost, prolongs survival and slows the course of the disease. It saves time in cases where the decision for lung transplantation needs to be made quickly.

 

Do doctors have sufficient knowledge and awareness about this disease?

• Although it is taught as a course in medical faculties, this disease can be easily overlooked within the general internal notion. In cases with progressive shortness of breath and cough; The presence of Velcro rales, honeycomb appearance and clubbing findings strongly suggest IPF in the diagnosis. These findings should be reminded to internal physicians in in-service training seminars and handbooks. Pulmonary eremboli (clot in the lung) is the most frequently responsible for exacerbations in diagnosed cases. (pneumonia), pneumothorax (air accumulation in the lung membrane) and heart failure should also be considered during the course of this disease. For this reason, it would be beneficial to refer cases to chest diseases physicians and review the diseases responsible for exacerbations.

What should be done to increase awareness about the disease?

• A male patient over the age of 50 who complains of shortness of breath, cough and smokes or has smoked in the past; If there are velcro rales and honeycomb findings on high-resolution tomography, IPF should be considered in the diagnosis until proven otherwise. Physicians can be informed about this issue by preparing posters, and patients can be informed by preparing a public service announcement.

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