Idiopathic fibroziruyushchy alveolitis

Description:

Alveolitis of fibroziruyushchiya idiopathic (synonyms: fibrosis is pulmonary idiopathic, Hammena-Rich's syndrome) – the progressing diffusion bilateral defeat of alveoluses and intersticial pulmonary fabric which is followed by development of diffusion fibrosis and the increasing respiratory insufficiency. The Fibroziruyushchy alveolitis has exclusively pulmonary localization, badly gives in to therapy, often terminates in a lethal outcome. The Fibroziruyushchy alveolitis – rather rare disease of not clear etiology, however tends to increase.

The Fibroziruyushchy alveolitis strikes men more often 50 years (20 cases from 100 thousand), than women are more senior (13 cases from 100 thousand). The lethality at a fibroziruyushchy alveolitis reaches 3,3 cases on 100 thousand population.

Symptoms of the Idiopathic fibroziruyushchy alveolitis:

Development of a fibroziruyushchy alveolitis gradual with development of irreversible changes in alveoluses and steady increase of an asthma. A long time patients do not attach it significance and do not see a doctor, explaining changes in the health with fatigue at work, the termination of sports occupations, a set of weight etc. Usually from the beginning of the first symptoms of a fibroziruyushchy alveolitis to the appeal to medical institution passes from 3 months to 1-3 years.

Serve as the leading complaints at a fibroziruyushchy alveolitis the expressed asthma at the minimum exercise stresses and unproductive cough. Patients are disturbed by the thorax pains (under shovels) interfering a deep breath, weight loss, weakness, muscular and joint pains, the increased body temperature. At a half of patients with a fibroziruyushchy alveolitis nail phalanxes take a characteristic form of "drum sticks" with "hour glasses". Cyanosis and short wind of a rezistentna to the carried-out therapy. Further there is an increase of an anoxemia, pulmonary hypertensia and right ventricular insufficiency.

In an end-stage of a fibroziruyushchy alveolitis signs of respiratory insufficiency and development of a pulmonary heart amplify: gray-ashy diffusion cyanosis, swelling of veins of a neck, hypostases, development of a cachexia.

Reasons of the Idiopathic fibroziruyushchy alveolitis:

Origins of a fibroziruyushchy alveolitis are not clear. There are assumptions of the autoimmune nature of a disease, the virus nature (герпесвирус, a hepatitis C virus, adenoviruses, a cytomegalovirus), genetic predisposition.

In prevalence of a fibroziruyushchy alveolitis professional, ecological, household and geographical factors matter. So, it is noticed that the farmers breeding birds, the workers contacting to wood, asbestos, metal and silicate dust, the smoking patients are most subject to development of an idiopathic fibroziruyushchy alveolitis. The inflammatory phenomena in alveoluses cause an irreversible thickening of their walls and decrease in permeability for gas exchange.

Development of an idiopathic fibroziruyushchy alveolitis is characterized by three interconnected processes: intersticial hypostasis, intersticial inflammation (alveolitis) and intersticial fibrosis.

In an acute stage of intersticial hypostasis there is a defeat of alveolar capillaries and an epithelium, their hypostasis and formation of the hyaline and membrane complexes interfering expansion of alveolar fabric at breath. At this stage regress of process or development of intersticial pneumonia is possible.

The chronic stage of an intersticial inflammation is characterized by further progressing of process, adjournment in alveoluses of collagen and development of widespread fibrosis with extensive damage of pulmonary fabric.

In an end-stage of intersticial fibrosis there is full substitution of a capillary network and alveolar fabric fibrous to formation of band expansions. Tissue of a lung reminds bee cells by outward.

Irreversible changes in an alveolar kapilyarnoy to system of lungs at a fibroziruyushchy alveolitis lead to restrictive changes, disorder of gas exchange, progressing of respiratory insufficiency and to death of the patient.

Treatment of the Idiopathic fibroziruyushchy alveolitis:

The purpose  of the treatment of a fibroziruyushchy alveolitis which is carried out by the pulmonologist is simplification of a course of a disease, delay of progressing of fibrosis of pulmonary fabric, high-quality improvement of life of the patient.

The special rehabilitation programs including physical trainings, psychological support of patients belong to non-drug treatment of a fibroziruyushchy alveolitis. The oxygen therapy reducing an asthma and increasing endurance of exercise stresses at patients with a fibroziruyushchy alveolitis has the expressed therapeutic effect. For the purpose of the warning of viral infections to all patients with a fibroziruyushchy alveolitis anti-influenza and anti-pneumococcal vaccination is shown.

For achievement of a condition of remission include antiinflammatory (glucocorticosteroids) in medicamentous therapy of a fibroziruyushchy alveolitis and anti-fibrous (Penicillaminum, colchicine) drugs, immunodepressants (Azathioprinum), and also their combination. Potassium drugs, a pyridoxine, bronchial spasmolytics are at the same time appointed верошпирон. Medicamentous therapy gives effect only in cases of lack of the expressed pneumofibrosis.

Radical treatment of a fibroziruyushchy alveolitis provides the transplantation of lungs giving high percent of 5-year survival – to 50-60%. Serve as indications to change of lungs expressed an anoxemia, диспноэ, decrease in ZhEL is lower than 70%, decrease in diffusion ability of lungs.