Idiopathic Fibrosing Alveolitis - Symptoms, Treatment, Forms, Stages, Diagnosis

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

Idiopathic fibrosing alveolitis

The content of the article:

1. Causes and risk factors
2. Forms of the disease
3. Disease stages
4. Symptoms
5. Diagnostics
6. Treatment
7. Possible complications and consequences
8. Forecast
9. Prevention

Idiopathic fibrosing alveolitis (Hammen-Rich syndrome, interstitial diffuse pulmonary fibrosis, fibrous pulmonary dysplasia, Scadding's syndrome) is a pulmonary disease characterized by diffuse damage to the interstitial tissue of the lungs, leading to the development of pneumosclerosis, pulmonary heart disease and respiratory failure.

Idiopathic fibrosing alveolitis - diffuse damage to the interstitial tissue of the lungs

In the pathological mechanism, several interrelated processes are distinguished:

• swelling of the interstitial tissue;
• alveolitis (inflammation of the alveoli);
• interstitial fibrosis.

The disease is rare - 8-10 cases per 100,000 people. However, in recent years, there has been a slight increase in the incidence of idiopathic fibrosing alveolitis. This is due to both a true increase in the incidence of the disease and an improved diagnosis of this lung pathology.

Causes and risk factors

Currently, the exact causes of idiopathic fibrosing alveolitis have not been established. It is assumed that the following factors may play a role:

• hereditary predisposition;
• infection with certain viruses (cytomegalovirus, adenovirus, hepatitis C virus, herpes simplex virus);
• autoimmune disorders.

It is known that some geographic, household, environmental and occupational factors can also affect the incidence. Idiopathic fibrosing alveolitis is more commonly diagnosed in the following populations:

• smokers;
• workers in contact with silicate, metal, asbestos or wood dust;
• bird farmers.

Forms of the disease

According to the characteristics of the clinical course, the following forms of idiopathic fibrosing alveolitis are distinguished:

• nonspecific interstitial pneumonia;
• acute interstitial pneumonia;
• desquamative interstitial pneumonia;
• common interstitial pneumonia.

Pathogenesis of idiopathic fibrosing alveolitis

Disease stages

In accordance with the characteristics of the inflammatory process, there are three stages of idiopathic fibrosing alveolitis:

1. Sharp. Damage to the epithelium and alveolar capillaries is observed, hyaline-membrane formations are formed, which do not allow the tissue of the alveoli to expand freely during inhalation.
2. Chronic. Collagen is deposited in the alveoli, and the interstitial tissue is replaced by fibrous tissue.
3. Terminal. Fibrous tissue almost completely replaces the tissue of the alveoli and capillaries. Multiple cavities appear in the lungs, as a result of which they resemble a honeycomb. Gas exchange is significantly impaired, respiratory failure is increasing, which ultimately leads to the death of the patient.

Symptoms

Idiopathic fibrosing alveolitis develops gradually and rather slowly. The first symptom of the disease is shortness of breath. At first, it is expressed slightly and occurs only against the background of physical exertion. At this stage, patients usually do not go to the doctor, believing that the malaise is associated not with the disease, but some other reasons (overweight, overwork). According to statistics, from the moment the first signs of the disease appear to the visit to a pulmonologist, it usually takes from 3 to 24 months. By this time, shortness of breath occurs already with minimal exertion, and sometimes even at rest. Other symptoms of the disease appear:

• unproductive cough;
• chest pain that prevents you from taking a deep breath;
• severe general weakness;
• losing weight;
• increased body temperature;
• joint and muscle pain;
• characteristic changes in the nail phalanges (symptom of drumsticks);
• cyanosis of the skin and mucous membranes.

Shortness of breath is the first sign of idiopathic fibrosing alveolitis

In the terminal stage of idiopathic fibrosing alveolitis, the manifestations of chronic respiratory and right ventricular heart failure rapidly progress in patients:

• swelling;
• swelling of the veins of the neck;
• diffuse cyanosis;
• cachexia.

At this stage of the disease, pulmonary edema often occurs.

Diagnostics

Diagnosis of idiopathic fibrosing alveolitis begins with a history and physical examination of the patient. At auscultation of the lungs and heart, attention is drawn to:

• muffled heart sounds;
• tachycardia;
• dry wheezing in the lungs with hard breathing;
• cellophane crackle symptom (crepitus).

To confirm the diagnosis, an examination is carried out, the program of which includes:

• general and biochemical blood test;
• radiography;
• computed tomography;
• spirometry;
• peak flowmetry;
• electrocardiography;
• bronchoscopy;
• open lung biopsy followed by histological analysis of the material obtained.

Treatment

The main goals of therapy for idiopathic fibrosing alveolitis are:

• slowing down the process of fibrous replacement of the interstitial tissue of the lungs;
• improving the quality of life of patients.

The treatment regimen includes:

• anti-inflammatory drugs (corticosteroids, cytostatics);
• antioxidants;
• antifibrotic drugs (colchicine, penicillamine);
• bronchodilators;
• anticoagulants;
• oxygen therapy.

Drug treatment improves the patient's condition

The indications for lung transplantation are:

• decrease in lung capacity below 70%;
• disturbances in the rhythm and frequency of breathing;
• severe hypoxemia;
• decreased diffuse lung capacity.

Possible complications and consequences

The main complications of idiopathic fibrosing alveolitis:

• pulmonary hypertension;
• respiratory failure;
• frequent bacterial pneumonia;
• lung cancer.

Forecast

The prognosis for idiopathic fibrosing alveolitis is always serious. The disease progresses steadily, leading to the death of patients. In the absence of appropriate therapy, death occurs 3-4 years after the onset of the first symptoms of the disease. With a good response to ongoing therapy, life expectancy increases to 10 years. The 5-year survival rate after lung transplantation is 50-60%.

Prevention

Prevention of idiopathic fibrosing alveolitis consists of the following measures:

• to give up smoking;
• timely and adequate treatment of viral infections;
• prevention of prolonged contact with occupational hazards (for example, silicates, asbestos or metal dust).

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Elena Minkina Doctor anesthesiologist-resuscitator About the author

Education: graduated from the Tashkent State Medical Institute, specializing in general medicine in 1991. Repeatedly passed refresher courses.

Work experience: anesthesiologist-resuscitator of the city maternity complex, resuscitator of the hemodialysis department.

The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!