Respiratory regulation at rest and under the influence of increasing hypercapnia was investigated in patients with chronic nonspecific pulmonary diseases (CNPD) with restrictive and obstructive types of lung ventilation capacity disorder. An increase in the inspiratory activity of the central mechanisms of respiratory regulation was revealed even at the early stages of disease. The determination of this type of activity in comparison with respiratory pattern parameters could be of diagnostic value. CNPD patients with congenital immunopathies (Kartagener-Sievert syndrome, CNPD with alpha 1 antitrypsin deficiency) demonstrated concomitant respiratory regulation disorders in the form of low inspiratory activity in response to hypercapnia suggesting a complicated course of disease in these patients.