The clinical manifestation of primary immunodeficiencies with a defect in antibody formation can be caused not only by severe long-term infectious lesions, but also by autoimmune diseases.
Twenty-two patients were diagnosed with primary a- (hypo)gammaglobulinemia. The course of the disease of the 15 patients was manifested by frequent infections (infectious phenotype, IP). Two people were diagnosed with Crohn's disease, five patients - rheumatoid arthritis (autoimmune phenotype, AP).
A comparison of the parameters of immunity showed that in patients with AP, the number of T cells expressing the HLA DR more (AP -18 ± 2.1%, IP - 9 ±1.7%), and the number of T-reg is less than of patients with IP (AP 0.6 ± 0.3%, IP-1.1 ± 0.6%), which also demonstrates index Treg/СD4: AP-1.5 ± 0.4%, IP-3.7 ± 0.8%. In the conditions of the autoimmune manifestation, in comparison with infectious, expression of TLR2 (AP-80 ± 5%, IP-42 ± 3%), TLR4 (AP-60 ± 5%, IP-21 ± 7%), HLA DR (AP -80 ± 7%, IP-40 ± 4%) with monocytes, also the synthesis of IL-6 (AP-14 ± 2.6 pg / ml, IP-2.6 pg / ml), IFN-γ (AP -93 ± 5.9 pg / ml, IP - 78 ± 8.3 pg / ml) increases, while the serum content of IL-17 (16 ± 3.3 pg / ml) was determined only in patients with AP
The clinical manifestation of autoimmune processes of patients with a-(hypo)gammaglobulinemia is associated with weakening of T-immunosuppression, increasing of the activational indexes of T-lymphocytes and antigen-presenting cells, increasing of the production of proinflammatory mediators