Idiopathic fibrosing alveolitis , diffuse interstitial pneumofibrosis , Hamman-Rich syndrome - a disease characterized by diffuse lesions of pulmonary interstitium followed by the development of pneumosclerosis and respiratory failure, hypertension of the pulmonary circulation and pulmonary heart. It is relatively rare (about 7-10 cases per 100 thousand people), autoimmune disorders (type III hypersensitivity, reactions of immune complexes) are of great importance in the development of this form. Serous-fibrinous impregnation of the alveolar septa, accumulation in the alveoli of exudate rich in fibrin , macrophages and especially neutrophils, pronounced proliferation of connective tissue and its sclerosis , hyaline membranes in the alveoli are characteristic. The lungs are dense, red-brown in color with a network of grayish stripes and foci of bullous emphysema. [2]
| HR syndrome | |
|---|---|
| ICD-10 | J 84.1 |
| ICD-10-KM | |
| ICD-9 | 516.3 |
| ICD-9-KM | |
| Omim | 178500 |
| Diseasesdb | 4815 |
| Mesh | D011658 |
Content
Clinic
The disease most often occurs in patients over the age of 50 years. The prevalence of the disease in men is noted, the sex ratio is approximately 1.7: 1 in favor of men (Johnston et al., 1997). The main complaints of patients are shortness of breath and unproductive cough. As the disease develops, there is an increase in shortness of breath, up to a complete disability of the patient: due to shortness of breath, the patient is not able to utter a phrase, sentence, cannot walk, serve himself. The onset of the disease is usually imperceptible, although sometimes patients describe the debut of ELISA as an acute respiratory disease, which suggests the role of viral infection in the genesis of the disease (Egan et al., 1997). Since the disease progresses rather slowly, patients manage to adapt to their shortness of breath, gradually reducing their activity and moving to a more passive lifestyle. Most patients at the time of the examination have a history of the disease lasting up to 1-3 years, and almost never - less than 3 months (du Bois and Wells, 2001). A productive cough is sometimes noted (up to 20%), even purulent sputum production, especially in patients with severe forms of ELISA. Fever is not characteristic of ELISA. Hemoptysis is also not characteristic of ELISA, and the appearance of this symptom in a patient with ELISA should guide the doctor towards the search for a lung tumor, which is 4–12 times more common in patients with ELISA compared to people in the general population, even after taking the smoking history (Hubbard et al. , 2000). Other symptoms may include general weakness, arthralgia, myalgia, a change in the nail phalanges in the form of "drumsticks" (up to 70%).
A characteristic auscultatory phenomenon in ELISA is end-inspiratory crepitus, which is compared with a "crack of cellophane" or a zipper ("Velcro" rales). Compared with crepitus in other diseases (pneumonia, bronchiectasis, congestion in the lungs), crepitus with ELISA is more delicate (fine crackles): less loud and higher in frequency, it is heard at the height of inspiration, i.e. in the course of the inspiratory period (Piirila et al., 1995). Most often, wheezing is heard in the posterior basal parts, although with the progression of the disease, crepitus can be heard over the entire surface of the lungs and during the entire phase of inspiration. In the early stages of the disease, basal crepitus may weaken or even completely disappear with the patient leaning forward (du Bois and Wells, 2001). Dry rales can be heard in 5-10% of patients and usually appear with concomitant bronchitis. Up to 50% of all patients have tachypnea.
As the disease progresses, signs of respiratory failure and pulmonary heart appear: diffuse gray ash cyanosis, increased tone II over the pulmonary artery, tachycardia, S3 gallop, cervical vein swelling, peripheral edema. The decrease in body weight of patients, up to the development of cachexia, is a characteristic sign of the terminal stage of ELISA.
Diagnostics
On the radiograph - strengthening and deformation of the pulmonary pattern, retina due to proliferation of connective tissue around the pulmonary lobules (picture of the “cellular lung”). There is a symptom of "Matt lung" (gentle homogeneous darkening of the pulmonary fields), more often in the lower parts of the lungs and subpleural. More sensitive diagnostic methods are scanning with gallium-67 and bronchoalveolar lavage ( bronchoscopy with rinsing of the bronchi and subsequent examination of the rinsing fluid). [3]
Treatment
The treatment effect is high in the initial stages of the disease, and decreases as fibrosis develops. Glucocorticoids are prescribed, with their insufficient effectiveness and further progression of the process, immunosuppressants (cyclophosphamide or azathioprine), penicillamine are added. Doses of drugs are reduced gradually, not earlier than 1.5 - 3 months after the start of treatment. Antibiotics are ineffective. In acute forms, the use of plasmapheresis is possible. However, the effectiveness of plasmapheresis in this pathology has not been studied. Plasmapheresis is recommended with removal of 25-30% of the volume of circulating plasma, with replacement by crystalloid infusion solutions. The course of treatment with plasmapheresis - 3 operations with an interval of 5-7 days between sessions.
Forecast
The prognosis in the absence of treatment is unfavorable - patients die after 4 to 6 years from the onset of the disease, and sometimes even after 1 to 2 months. Timely initiated treatment helps not only save lives, but also maintain the ability to work of patients with ELISA, which, however, must be under the supervision of a pulmonologist and rheumatologist for life.
Additional Facts
From this disease on January 28, 2017, at the 65th year of his life, the Soviet and Belarusian musician, the former lead singer of VIA " Verasy " Alexander Tikhanovich , died.
Notes
Literature
- “Diseases of the respiratory system”, a 4-volume manual for doctors, edited by Academician of the Academy of Medical Sciences of the USSR N.R. Paleev , Moscow, Medicine, 1989 ISBN 5-225-01646-4