The interplay between T- and B-cell compartments during naïve, effector and memory T cell maturation is critical for a balanced immune response. Primary B-cell immunodeficiency arising from X-linked agammaglobulinemia (XLA) offers a model to explore B cell impact on T cell subsets, starting from the thymic selection. Here we investigated characteristics of naïve and effector T cell subsets in XLA patients, revealing prominent alterations in the corresponding T-cell receptor (TCR) repertoires. We observed immunosenescence in terms of decreased diversity of naïve CD4+ and CD8+ TCR repertoires in XLA donors. The most substantial alterations were found within naïve CD4+ subsets, and we have investigated these in greater detail. In particular, increased clonality and convergence, along with shorter CDR3 regions, suggested narrower focused antigen-specific maturation of thymus-derived naïve Treg (CD4+CD45RA+CD27+CD25+) in the absence of B cells - normally presenting diverse self and commensal antigens. The naïve Treg proportion among naïve CD4 T cells was decreased in XLA patients, supporting the concept of impaired thymic naïve Treg selection. Furthermore, the naïve Treg subset showed prominent differences at the transcriptome level, including increased expression of genes specific for antigen-presenting and myeloid cells. Altogether, our findings suggest active B cell involvement in CD4 T cell subsets maturation, including B cell-dependent expansion of the naïve Treg TCR repertoire that enables better control of self-reactive T cells.

Hereditary angioedema (HAE) due to of C1-inhibitor deficiency is a rare disease, which causes a strong physical and psychological impact on patients’ lives. HAE affects daily life, career opportunities, and social activity. In addition, fear of the edema development, as well as the associated with it pain and risk of death leads to depressive disorders specific for chronic (especially life-threatening) diseases. The emergence of modern pathogenetic drugs can minimize the effect of HAE and improve the patients’ quality of life. There are three different therapeutic strategies that are used for HAE treatment: long-term prophylaxis, short-term and on-demand therapy for acute attacks. This article focuses on long-term prophylaxis.

The emergence of highly effective therapy has led to an increase in the life expectancy of patients with primary immunodeficiency (PID). Currently, we have the opportunity to observe the first generation of adults suffering from PID. Objective. To study the structure of the PID forms in Russian adults of the register of Institute of immunology of FMBA Russia. Materials and methods. The analysis of PID structure, demographic data and need for therapy was carried out on the basis of472 patient’s case histories who were treated in the Institute of immunology from 1983 to 2017. Results. The main part of patients older than 18 years (472 people) of Russian PID population are presented with predominant antibody deficiency - 61,22% and hereditary angioedema (HAE) - 30,72%, other PID forms are presented by isolated clinical cases. In 21,82% of cases, the disease debuted at the age of 18 years, with the diagnosis of PID was established after 18 years in 62,4% of cases (294 patients out of472). The average age of adult registry of PID patients is 39,6±6 years, median age - 35,6 years, maximum age - 86 years. Persistent disability is observed in 12,5% of cases. A study of the availability and adequacy of therapy showed that replacement therapy with immunoglobulins (IVIG) in need by 53,7%, but only 27,4% have it on the regularly and conditionally adequate dose, for 5,7% IVIG therapy is unavailable, while the majority of patients - 66,8% receive IVIG irregular and/or in inadequate dose. The presence of splenomegaly, hepatomegaly and enteropathy significantly lengthen the diagnosis period (p

Chronic urticaria (CU) is an urgent medical and social problem. The instruments of everyday clinical practice are international and domestic guidelines which briefly reflect modern ideas about various aspects of CU. Recently, a new opportunity has emerged to expand theoretical and practical experience in the management of patients with CU, to carry out educational activities to disseminate modern knowledge about urticaria, to conduct research in the field of studying the pathogenesis, treatment, and prevention of exacerbations of the disease. This opportunity is realized through the centers of excellence for working with such patients (GALEN UCARE centers). One of the five Russian centers, have prepared and conducted an online training program "Chronic urticaria: scientific and medical achievements and practical aspects of patient management." at the Federal State Budgetary Institution State Research Center "Institute of Immunology" FMBA of Russia. The event was held by the employees of the Federal State Budgetary Institution State Research Center "Institute of Immunology" of the FMBA of Russia on November , 2020.

Angioedema is caused by a local increase in vascular permeability induced by bradykinin or mast cell mediators. The main challenge of the differential diagnosis of angioedema lies in the fact that different pathophysiological processes may lead to a very similar clinical picture. Moreover, verification of the type of angioedema is crucial for selection of appropriate therapy. In 2020, a school for doctors Chronic urticaria: scientific and medical accomplishments and practical aspects of patient management was held on the basis of the National Research Center Institute of Immunology of the FMBA of Russia as part of the work of the reference center that provides expert assistance to patients with urticaria (GALEN UCARE). A clinical case series of related patients with hereditary angioedema due to a mutation in the PLG gene was presented to demonstrate the challenges of the differential diagnosis between types of angioedema. This article focused on the description of this series.