ALPHA-1 PROTEINASE INHIBITOR FOR DELAY OF BEGINNING OR PROGRESS OF PULMONARY EXACERBATIONS Russian patent published in 2017 - IPC A61M15/00 A61K38/55 A61P11/00 

FIELD: medicine.

SUBSTANCE: methods are proposed comprising administration of an effective amount of an alpha1 proteinase inhibitor purified by chromatography (A1PI-HC) to the subject by daily inhalation for a long-term period of time (from 21 days to 10 years). For one version of the method, A1PI-HC is aerosolized and administered using an inhaler in an effective amount of A1PI-HC from about 25 to about 750 mg per day, or from about 0.5 to about 15 mg/kg/day. The patient's age should be at least 12 years. Pulmonary disorder or disease is fibro-cystic degeneration, chronic obstructive pulmonary disease (COPD), deficiency of alpha1 proteinase (A1PI deficiency), emphysema, asthma, mycobacterial infection, pneumonia, bronchiectasis or chronic bronchitis. A version of the method including preliminary identification of subjects with an increased risk of pulmonary diseases exacerbations acceptable for A1PI maintenance therapy is also proposed. The method includes evaluating one or more subject criteria, such as (a) age; (b) history of exacerbations; (c) lungs function (FEV₁); (d) chronic productive cough (with phlegm); (e) infectious load by the upper and lower respiratory tract pathogen; (f) inflammation markers of the respiratory tract of exhaled gas; (g) response to exogenous provocation for airways hyperreactivity research; (h) number and classes of concomitant medications; (i) the profile of genetic risk for respiratory disease; (j) environmental risk factors, such as smoking history, allergies, occupational risk factors and/or air pollution effects. It is then determined whether or not the subject is a candidate for A1PI maintenance therapy based on the above criteria, and an effective amount of A1PI-HC is administered to the subject by daily inhalation for a long-term period from 21 days to 10 years. For another version of the method, the above-mentioned inhaled administration of A1PI-HC is performed using an aerosol generated by the inhaler one or more times per day. The cumulative effective dose of A1PI-HC is from about 25 to about 750 mg once a day.

EFFECT: group of inventions provides effective prevention and treatment of pulmonary diseases and their exacerbations associated with alpha1-proteinase deficiency due to drug administration directly into the lower parts of the respiratory tract at low therapeutic doses.

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