Fibrosing alveolitis - causes, symptoms, diagnosis and treatment
Fibrosing alveolitis - Pathological process, which is based on an extensive loss of interstitial lung tissue, leading to the development of fibrosis and respiratory failure. The progression of fibrosing alveolitis is accompanied by a steady increase of weakness, weight loss, shortness of breath, unproductive cough, chest pain, cyanosis. Diagnosis is based on data from X-ray and computed tomography lung, spirometry, lung biopsy. Treatment of fibrosing alveolitis include anti-inflammatory and immunosuppressive therapy, oxygen therapy; on the testimony - lung transplantation.
Fibrosing alveolitis
herpes virus, hepatitis C virus, adenovirus, cytomegalovirus), hereditary predisposition.
The prevalence of fibrosing alveolitis relevant occupational, environmental, consumer and geographical factors. Thus, it is seen that the development of idiopathic fibrosing alveolitis are most susceptible poultry farmers, workers in contact with the wood, asbestos, metal and silicate dust, smoking patients. Inflammation in the alveoli causes irreversible thickening of their walls and reduction of permeability for gas exchange.
The development of idiopathic fibrosing alveolitis is characterized by three interrelated processes: interstitial edema, interstitial inflammation (alveolitis) and interstitial fibrosis.
In the acute stage of interstitial edema occurs loss of alveolar capillaries and epithelium, the formation of edema and hyaline membrane complexes that impede the expansion of alveolar tissue breathing. At this stage of the process is possible or promoting regression of interstitial pneumonia.
Chronic interstitial inflammation stage is characterized by the further progression of the process, deposition of collagen in the alveoli and the development of advanced fibrosis with extensive damage to lung tissue.
In end-stage interstitial fibrosis is a full replacement of the capillary network and alveolar tissue with the formation of fibrous cavity extensions. lung tissue reminiscent in appearance to a honeycomb.
Irreversible changes in alveolar-capillary pulmonary system with fibrosing alveolitis lead to restrictive changes, breakdown of gas exchange, the progression of respiratory failure and patient death.
Pulmonology allocate 3 forms of fibrosing alveolitis:
Fibrosing alveolitis, idiopathic include morphological forms: the usual interstitial pneumonia, desquamative interstitial pneumonia, acute interstitial pneumonia and non-specific interstitial pneumonia.
By alveolitis exogenous allergic include diseases caused by prolonged and intense inhalation of dust containing animal and vegetable proteins, or inorganic pollutants, and accompanied by diffuse allergic lesions of interstitial and alveolar lung structures (syndromes "light farmer", "easy fanciers", "mild dairies" "light worker" and so on. d.).
Fibrosing alveolitis include toxic lung disease resulting from the toxic effects of industrial, chemicals and drugs of chemotherapy.
Adrift distinguish acute, subacute and chronic fibrosing alveolitis.
joint pain, fever. Half of the patients with fibrosing alveolitis nail phalanxes become the characteristic form of "clubbing" with the "hour-glass". Cyanosis and dyspnea refractory to therapy. In the future, there is an increase of hypoxemia, pulmonary hypertension and right ventricular failure.
In the terminal stage of fibrosing alveolitis increasing signs of respiratory failure and the development of pulmonary heart: gray-ash diffuse cyanosis, swelling of the neck veins, edema, cachexia development.
Complications fibrosing alveolitis
Techeneie fibrosing alveolitis causes progressive respiratory failure, pulmonary heart development, may be complicated by pulmonary edema.
tachycardia and muted tones.
When X-ray picture of lungs idiopathic fibrosing alveolitis match bilateral diffuse small focal changes in the lungs, more pronounced in the lower divisions, emphysema. Late stage fibrosing alveolitis correspond secondary cystic changes in the lungs ( "honeycomb lung"). Dynamic Radiography provides information about the irreversibility of the changes characteristic of fibrosing alveolitis.
Computed X-ray diagnostics allows to detect changes in the lungs in the early stages. A characteristic feature of fibrosing alveolitis is the definition of the symptom "frosted glass" - a gentle homogeneous darkening lung fields. According to CT pulmonary possible to determine the portion of the modified fabrics for taking lung biopsy.
Indicators of respiratory function (spirometry, peak flow meter) with fibrosing alveolitis show ventilation violations and reducing the diffusion capacity of the lungs.
Laboratory blood parameters are characterized by leukocytosis and accelerated ESR, increased hemoglobin and hematocrit; increased levels of fibrin, sialic acid, immunoglobulin, determination of rheumatoid factor.
The ECG - signs of hypertrophy and overload of the right heart.
Bronchoscopy with fibrosing alveolitis conducted to differential diagnosis of infectious or neoplastic processes in the lungs. The obtained during bronchoscopy lavage fluid is determined by the increase of total protein, immunoglobulin, cytoplasmic inclusions with macrophages, neutrophils.
Holding an open lung biopsy with fibrosing alveolitis to determine the stage of the disease, the selection of appropriate treatment, the disease development. Histologically, there are five degrees of change:
Fibrosing alveolitis should be differentiated from pneumonia, granulomatosis, sarcoidosis, disseminated tuberculosis, bronchioloalveolar cancer, pneumoconiosis, diffuse amyloidosis and others.
It worked out great and small diagnostic criteria fibrosing alveolitis. Since most likely fibrosing alveolitis can be diagnosed by determining the larger 4 and 3 minor criteria.
More criteria verification idiopathic fibrosing alveolitis are:
For small criteria idiopathic fibrosing alveolitis include:
pulmonologist treatment of fibrosing alveolitis is to facilitate the course of the disease, slowing the progression of fibrosis of the lung tissue, improving patient quality of life.
For non-drug treatment of fibrosing alveolitis are special rehabilitation programs that include physical training, psychological support to patients. Pronounced therapeutic effect has oxygen therapy, reduces shortness of breath and increases endurance of physical activity in patients with fibrosing alveolitis. In order to prevent viral infections in all patients with fibrosing alveolitis shown influenza vaccination and antipnevmokokkovaya.
In order to achieve the state of remission in medical therapy include anti-fibrosing alveolitis (steroids) and anti-fibrotic (penicillamine, colchicine) drugs, immunosuppressants (azathioprine), and a combination thereof. At the same time appointed veroshpiron, potassium supplements, pyridoxine, bronchodilators. Drug therapy has the effect only in cases where no pronounced fibrosis.
Radical treatment of fibrosing alveolitis involves transplantation of the lungs, which gives a high percentage of 5-year survival rates - up to 50-60%. The indications for lung transplantation are pronounced hypoxemia, dyspnea, decreased vital capacity below 70%, the reduction of diffuse lung capacity.
Prediction and prevention in fibrosing alveolitis
During idiopathic fibrosing alveolitis after the drug therapy observed periods of remission, but the disease still progresses gradually. The average survival rate in the diagnosis of untreated fibrosing alveolitis is 3-4 years.
A more favorable course of different toxic and allergic alveolitis, which in troubleshooting a provoking factor in the early stages may regress.
Measures of prevention of idiopathic fibrosing alveolitis include preventing infection, exclusion of harmful occupational, environmental and consumer factors. Patients with fibrosing alveolitis should be followed up by a pulmonologist, allergist and physician-pathologist.