Alveolar proteinases is the pulmonary pathology connected with hyperproduction of surfactant and filling of a cavity of alveoluses with excess proteinaceous and lipidic weight. In case of alveolar proteinase steadily progresses short wind, there is unproductive cough, subfebrilny fever, weight loss, perspiration, bystry fatigue can be noted, to develop respiratory insufficiency. Diagnostics is based on given to a X-ray analysis and a computer tomography of lungs, functions of external breath, a biopsy of easy, laboratory researches. Therapy alveolar proteinase consists in carrying out a medical bronkhoalveolyarny unleavened wheat cake.
Alveolar proteinases – the interstitsialny disease of lungs which is followed by changes of pulmonary fabric owing to adjournment on an internal surface of alveoluses of a proteinaceous and lipidic substratum. Prevalence of pathology is low – 0,2 cases on 1 million population. In most cases the disease demonstrates at the age of 30-50 years though it can meet at children and elderly patients. Among the diseased obvious prevalence of males is traced - the ratio of men and women makes 5:1. Alveolar proteinases can be primary (idiopathic) and secondary (to develop against the background of the available pathology), congenital or acquired. On a current in pulmonology differentiate a sharp and chronic form alveolar proteinase.
Alveolar proteinase the defining factors have various forms. Congenital alveolar proteinases it is caused by the gene mutations leading to pathology of pulmonary metabolism, in particular, to violation of synthesis of proteins of the surfaktantny B or C type, anomaly of a receptor to GM-KSF. The most often meeting idiopathic alveolar proteinases develops in the presence autoantitet to GM-KSF, causing damage of its structure and decrease in functional activity. Rare forms secondary alveolar proteinase are connected with deficiency and functional insufficiency of alveolar macrophages. Chronic viral or bacterial infections (especially, caused by pnevmotsist, mikobakteriya, mushrooms), smoking, professional harm (long inhalation of pollyutant by production of heavy metals, silicon, aluminum, plastic), gemoblastoza, decrease in immunity can be the cause of such state.
The proteinaceous and lipidic substance collecting in lungs consists of phospholipids of surfactant, the "foamy" alveolar macrophages (loaded with lipidic granules), eozinofilny grains, immunoglobulins and proteins of alveolar liquid. Pulmonary surfactant (an antiatelektatichesky factor) for 90% presented by lipidic fraction, and also proteins and a small number of polysaccharides sekretirutsya by cages of an epithelium of pulmonary sacks – alveolotsitam of the II type. Covering an internal surface of alveoluses in the form of a thin film, it regulates a superficial tension on border air-liquid, interfering with fall of alveoluses at breath. Surfactant promotes diffusion and digestion of oxygen, regulates water exchange in lungs, has bactericidal and immunomodulatory effect. The catabolism of surfactant is provided with a full cycle of recirculation in alveolotsita, and also its fagotsitozy alveolar macrophages. The granulotsitarno-makrofagalny koloniyestimuliruyushchy factor (GM-KSF) acts as a key factor of regulation of this process.
The mechanism of development alveolar proteinase is connected with hyperproduction of the changed surfactant, its incomplete absorption by alveolar macrophages, insufficiency GM-KSF. Capture of a large amount of surfactant alveolar macrophages leads to their degenerate changes and dysfunction that promotes the further progressing accumulation of excess of proteinaceous and lipidic substance in alveoluses.
Symptoms alveolar proteinase
In clinical development alveolar proteinase allocate 3 stages. In an initial I stage the expressed functional violations and clinical manifestations are absent, pathology comes to light at a planned radiological research. The II stage is characterized by emergence of respiratory insufficiency of the I degree, strengthening of radiological changes from lungs. The following, III stage is shown by the developed clinical picture, progressing of respiratory insufficiency to the II degree, accession of a secondary infection and bronchitis. In a terminal stage formation of pulmonary heart is possible.
The accruing short wind and cough act as the leading symptoms defining clinic alveolar proteinase. At first short wind develops only against the background of physical activity, then begins to disturb at rest. The nature of cough - unproductive with allocation of a poor phlegm, in the beginning – mucous, then – purulent. The blood spitting at alveolar to proteinase arises seldom. During the sharp period the subfebrilny temperature, an indisposition, perspiration, loss of weight, bystry fatigue, sometimes thorax pains is noted. At a long current cyanosis as manifestation of respiratory insufficiency, a symptom of "Hippocrates's fingers" can be noted.
At fizikalny inspection over the lower departments of lungs the weakened vesicular breath and an insignificant krepitation is listened, shortening of a perkutorny sound comes to light. Changes of blood are not specific - a politsitemiya, a γ-globulinemiya, increase in activity of LDG, high level of cholesterol, Ca, serumal surfaktantny proteins A and D. In a phlegm existence of SHIK+ of substances is defined. The analysis of gas composition of blood at expressed alveolar to proteinase indicates an arterial gipoksemiya; at an easy current it is observed only after physical activity.
The X-ray analysis and KT of lungs visualize bilateral disseminirovanny melkoochagovy shadows in basal and radical segments, inclined to merge, decrease in transparency (a phenomenon of "opaque glass"). At a terminal stage interstitsialny dystrophic and fibrous changes are noted. The spirometry reveals restrictive type of respiratory insufficiency, decrease in ZhYoL. The analysis of washing waters of bronchial tubes reveals their muddy shade, the increased protein content, CHIC-positive coloring, high level of T-lymphocytes, decrease in quantity of alveolar macrophages. The open, torakoskopichesky or chrezbronkhialny biopsy of lungs with histology of bioptat confirms presence of proteinaceous and lipidic exudate at alveoluses and surfactant plates in alveolar macrophages.
Alveolar proteinases differentiate from other disseminirovanny processes in lungs (tuberculosis, a sarkoidoz, radiation injuries of lungs, a fibroziruyushchy alveolit, bronkhoalveolyarny cancer, Gudpascher's syndrome, a leyomiomatoz, kollagenoz, a rheumatoid vaskulit), secondary proteinase, developed against the background of hematologic pathology (leukemias, lymphoma).
Treatment alveolar proteinase
At absence or insignificance of manifestations treatment alveolar proteinase can not be carried out. In case of the expressed clinical picture to reach improvement of the general state and carrying out a medical bronkhoalveolyarny unleavened wheat cake with physiological solution and medicines helps to provide long remission (heparin, trypsin, streptazy, atsetiltsisteiny). Use of system corticosteroids and immunodepressants does not render medical effect, but can increase risk of development of secondary infections. Antibiotic treatment is shown only in the presence of bacterial complications.
The Bronkhoalveolyarny unleavened wheat cake is carried out in the conditions of the general anesthesia and IVL. Lungs are washed out serially, to 15 times everyone. At further bystry accumulation of proteinaceous and lipidic complexes in alveoluses repeated medical procedures in 6-12-24 months are required. After the procedures of a bronkhoalveolyarny unleavened wheat cake improvement of clinical and functional indicators, positive dynamics of a radiological picture is observed. Transplantation of lungs is not expedient as it alveolar proteinases recurs in a transplanted organ.
Forecast and prevention
Current alveolar proteinase rather favorably. The disease progresses slowly, spontaneous remissions and recovery are possible, 5-year survival makes – 80%. Inadequate therapy, accession of a secondary superinfection significantly worsen the forecast. The lethal outcome at alveolar to proteinase is connected with development of heavy respiratory insufficiency and a decompensation of pulmonary heart. Prevention consists in an exception of smoking, influence of provocative professional and household factors. During remission patients with an alveolar proteinoz are observed at the pulmonologist.