Vol 15, No 4 (2018)

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<0,05). B-cell phenotyping showed that this method is a specific and reproducible test for the diagnosis of common variable immunodeficiency (CVID), allows to minimize the number of diagnostic errors and reduce the time of diagnosis. The search for reliable immunological markers-predictors of complications of CVID did not give results.

Background. Beta-lactams are the most widely used group of antibacterial agents. They are used for treating of infectious complications of varying severity, including urgent situations. The exclusion of the entire beta-lactam group not only leads to a significant increase in the cost of treatment, length of hospitalization and the formation of resistance of the flora to alternative antibiotics, but also may be life threatening in some cases. Therefore, correct diagnosis and establishement of the offending medication can prevent the development of allergic reactions and to avoid unreasonable lifelong bans on the use of certain medications, which often lead to a decrease of the effectiveness of treatment and increase its cost. The aim of the study was to develop the diagnostic and management algorithm and of of patients with presumptive diagnosis of delayed drug hypersensitivity to beta-lactams. Materials and methods. A comprehensive in-depth examination, including in vivo tests with beta-lactams was done in 53 patients (37 - 70% women, and 16 - 30% men) with a presumptive diagnosis of delayed drug hypersensitivity to beta-lactams. The minimum age at the time of the reaction was 4 years, the maximum age was 86 years, the average age was 37 years,the median was 38,5 years old. The minimum age at the time of testing was 6 years, the maximum age was 89 years, the average age was 44 years, median was 44 years old. Results. In the group of patients with delayed drug hypersensitivity to beta-lactams (n=21) the diagnosis was confirmed by skin testing in 19 (90,5%) patients, in 18 (85,7%) to aminopenicillins, and 1 (4,8%) to ceftriaxone. In 2 (9,5%) cases, the diagnosis was established using a drug provocation test: for amoxicillin (4,8%) and for ceftriaxone (4,8%). According to the obtained data, the specificity of skin tests for the diagnostics of delayed drug hypersensitivity to beta-lactams was 100%. In case of delayed drug hypersensitivity to aminopenicillins, the sensitivity of skin tests was 94,7%, the negative predictive value of skin tests was 93,7%, the overall accuracy of skin testing was 97%, and the incidence of false-negative results was 5,3%. Conclusion. Skin testing is promising and safe in the diagnositics of delayed drug hypersensitivity to beta-lactams. It allows to exclude unreasonable contraindications for the use of beta-lactams, thereby increasing the effectiveness of treatment while reducing economic costs.

Urticaria is a disease which symptoms are caused by the activation and degranulation of the mast cells (MC). Urticaria and angioedema persisting for more than 6 weeks is concerning to be the chronic form of the disease (CU). Chronic spontaneous urticaria (CSU) is the most common subtype of CU and has a great negative impact on all aspects of the patient’s life, causing a decreasing a quality of life, as a burden for a health system and society as a whole. In the present version of the conciliatory document diagnostic approaches in CSU have three goals: to make a differential diagnosis; to evaluate an activity of the disease, its control and the impact on the patient’s life; to identify the triggers of exacerbation or, if possible, the causes of a disease. A basic assessment of disease activity (UAS, AAS), quality of life (CU-Q2oL, AE-QoL) and disease activity control (UCT) are necessary for decisionmaking in the treatment of patients with CSU, better understanding of the disease burden, and documentation improvement and standardization. The aim of a treatment of patients with CSU is the complete control of the symptoms. A therapy should be performed by steps. On the first step a 2nd generation of Hj-antihistamines in standard doses has to be applied. In case of unresponsiveness a dose step-up up to 4 times is recommended, at the third step omalizumab is advised, at the fourth step (inefficiency of omalizumab) - cyclosporine. At any step, it is possible to use a short term course of glucocorticosteroids to set back an exacerbation.

An expert meeting «Place of Leukotriene receptor antagonists in treatment of Allergic Rhinitis» took place under the auspices of Russian Association of Allergists and Clinical Immunologists and Russian Rhinologic Society. Allergic rhinitis (AR) is an inflammatory disorder and it’s based on many cells and mediators release as well as CysLT. Montelukast is a selective leukotriene receptor antagonist, CysLT1 selective inhibitor (cysteine receptors of LTC4, LTD4, LTE4). Consensus document (Expert Statement LIRA (Leukotrienes Impact on Rhinitis Allergic) describes AR therapeutic treatment approaches for achieving AR symptoms control and patient adherence improvement.

В рамках состоявшегося в 2018 г. в Мюнхене (Германия) Конгресса Европейской Академии Аллергологии и Клинической иммунологии (EAACI) состоялся симпозиум компании «Сталлержен Грир», посвященный актуальным вопросам доказательной медицины с использованием данных из реальной клинической практики при проведении аллерген-специфической иммунотерапии. Членами президиума на симпозиуме были: Стефан Цилен [(Stefan Zielen) отделение аллергологии, пульмонологии и муковисцидоза, отделение для детей и подростков, Университетская больница им. Гёте, Франкфурт (Германия)] и паскаль Демоли [(Pascal Demoly) отделение пульмонологии и лекарственной зависимости, больница Арно де Вильнев, университет Монпелье, Монпелье, и университеты Сорбонны (Франция)]. С докладами выступили: Адам Фокс [(Adam Fox) «Гай и Сент-Томас нХС Фаундейшн Траст», лондон (Великобритания)], Стефан Цилен [(Stefan Zielen) отделение аллергологии, пульмонологии и муковисцидоза, отделение для детей и подростков, Университетская больница им. Гёте, Франкфурт (Германия)], паскаль Демоли [(Pascal Demoly) отделение пульмонологии и лекарственной зависимости, больница Арно де Вильнев, университет Монпелье, Монпелье, и университеты Сорбонны (Франция)], Ульрих Ван [(Ulrich Wahn) отделение педиатрической пульмонологии и иммунологии, Медицинский университет Шарите, Берлин (Германия)].