Vol 26, No 1 (2023)

Cover Page

Full Issue


Changes in mucosal immunity of oral cavity upon tooth loss in patients with periodontal diseases

Malyshev M.E., Kerimkhanov C.A., Iordanishvili A.K., Bumai A.O.


Partial or complete loss of teeth occurs in elderly and senile people, caused, mainly, by chronic generalized periodontitis. At the same time, the impact of presence or absence of persisting teeth and periodontium, is practically not covered in the literature as a factor of balance in the oral cavity, including local immunity of the mucous membranes. Our work concerned the changes in local immunity of the oral cavity occuring with the loss of natural teeth. We have observed 45 elderly people who were divided into 3 study groups, i.e., without inflammatory periodontal pathology (1), with periodontitis (2) and with chronic periapical inflammatory processes in the absence of periodontal inflammation (3). In order to sanitize oral cavity before the upcoming dental prosthetics, the patients of study groups 2 and 3 underwent extraction of all teeth in the upper and lower jaws. Indices of local immunity of the oral cavity in the salivary fluid of patients were assessed before surgical sanitation of the oral cavity (before the teeth extraction) and 30-35 days after removal of the last tooth. We have measured the salivary levels of secretory immunoglobulin A (sIgA) as well as pro-inflammatory cytokines, i.e., interleukin-1â (IL-1â), interleukin-6 (IL-6), interleukin-8 (IL-8), tumor necrosis factor-á (TNFá), and anti-inflammatory cytokines, e.g., receptor antagonist of interleukin-1 (RAIL), interleukin-4 (IL-4), interleukin-10 (IL-10)), as well as contents of antimicrobial peptides in saliva (catelicidin LL-37 and alphadefensins 1-3 (HNP1-3). We have found that the development of inflammation in severe inflammatory periodontal diseases, in particular, chronic generalized periodontitis requiring tooth extraction for oral cavity sanitation is characterized by functional insufficiency of secretory immunity of the oral mucosa associated with decreased secretion of secretory immunoglobulin A and antimicrobial peptides of neutrophilic origin, as well as a shift in the salivary cytokine balance towards increased production of pro-inflammatory cytokines. Removal of teeth, as the main source of inflammation and the basis for maintenance of dysbiotic microbiome biofilm leads to elimination of inflammation and the restoration of immune balance in the oral cavity.

Russian Journal of Immunology. 2023;26(1):7-16
pages 7-16 views

Features of mucosal immunity in the development of periodontal diseases in patients with type II diabetes mellitus

Markelova E.V., Golitsyna A.A., Yugai Y.V., Pervov Y.Y., Kovalchuk V.K.


Chronic generalized periodontitis takes one of the leading places in the structure of dental diseases. Inflammatory periodontal disease is one of the most common complications of diabetes. An important role in the progression of periodontitis in type II diabetes mellitus (T2DM) belongs to disorders of the immune system that affect the supportive/retaining complex of the tooth. Inflammation of periodontal tissues which occurs in the patients with carbohydrate metabolism disorders is characterized by a more severe course, which worsens the condition and quality of life of patients with T2DM thus leading to tooth loss. In this regard, the immunological aspects of developing periodontal pathology under conditions of carbohydrate metabolism disorders are of great interest. Purpose of our study included comparative assessment of local TNFá, TNFâ, IFNã, IL-1â, IL- 12, IL-17A levels in patients with periodontitis with and without carbohydrate metabolism disorders (type II diabetes mellitus), as well as in patients with type II diabetes mellitus without signs of periodontitis.

127 patients were examined, aged 30 to 59 years. The patients were divided into 3 groups, i.e., group I included patients suffering from periodontitis of varying severity without known comorbidities (47 persons); Group II consisted of patients with T2DM and periodontitis of varying severity (49 persons); Group III included patients with T2DM without symptoms of periodontitis (30 persons). The control group consisted of 30 practically healthy volunteers, matched to the patients for age and sex. Saliva specimens were used for laboratory studies. The levels of TNFá, TNFâ, IFNã, IL-1â, IL-12, IL-17А were determined by ELISA sandwich technique, with specific reagents purchased from R&D Diagnostics Inc (USA).

A significant increase in the IL-1â, TNFá, IFNã and IL-17A levels was found in patients of all groups compared with controls. At the same time, the increased concentrations of IFNã, IL-1â, IL-17A correlated with increase in periodontitis severity in the patients of both groups. Decreased TNFâ levels in saliva samples were revealed in the patients from almost all groups, regardless of the periodontitis severity. Significantly increased levels of IL-12 (p40 subunit) were recorded in saliva of the persons from group II and III when compared with controls and the group without diabetes mellitus.

Russian Journal of Immunology. 2023;26(1):17-26
pages 17-26 views

Cooperation of idiotypic and anti-idiotypic antibodies at the steroid-depended chemical carcinogenesis

Polenok E.G., Gordeeva L.A., Mun S.M., Kostyanko M.V., Antonov A.V., Verzhbitskaja N.E., Bairamov P.V., Kolpinskiy G.I., Vafin I.A., Glushkov A.N.


Immunological research of steroid-depended chemical carcinogenesis in humans is based on positive experience in the clinical usage of selective estrogen receptor modulators and experimental design of immunological methods for human protection from environmental carcinogens. Our study aimed for research of idiotypic antibodies against benzo[a]pyrene, estradiol and progesterone (IgA1-Bp, IgA1-E2 and IgA1-Pg), in connection with anti-idiotypic antibodies specific to estradiol and progesterone (IgG2-E2 and IgG2-Pg) in serum samples of postmenopausal healthy women (HW) and ER+/PR+ stage I breast cancer patients (BCP). Idiotypic antibodies were studied in 402 HW and 475 BCP using ELISA technique, with BP, E2 and Pg conjugated with bovine serum albumin as adsorbed antigens. The anti-idiotypic antibodies were studied using ELISA method and monoclonal antibodies against E2 and Pg as adsorbed antigens. High individual ratios of IgA1-Bp/ IgA1-Pg > 1 and IgA1-E2/IgA1-Pg > 1 were revealed in 42.1% and 48.5% HW, and in 71.1% and 78.1% of BCP cases (p < 0.0001, OR = 5.9 and OR = 3.8, respectively). High IgG2-E2 > 4 levels were found in 23.4% HW and in 41.2% of BCP group (p = 0.0001, OR = 2.3). Combination of IgA1-Bp/IgA1-Pg ≤ 1 with IgG2-E2 ≤ 4 and IgG2-Pg > 2 was more common in HW, than in BCP (29.3% vs 5.8%, p < 0.0001, OR = 0.1). Combinations of IgA1-Bp/IgA1-Pg > 1with IgG2-E2 > 4 or with IgG2-Pg > 2 were more frequent in BCP, than in HW (12.0% and 31.8% vs 4.9% and 15.2%, accordingly, p = 0.01, OR = 2.7 and p = 0.001, OR = 2.6), as well as combination of IgA1-Bp/IgA1-Pg > 1 with IgG2-E2 > 4 and IgG2-Pg > 2 (23.4% vs 9.8%, p = 0.0003, OR = 2.8). Similar specific features were found in HW and BCP when studying IgA1-E2/IgA1-Pg ratio with IgG2-E2 and IgG2-Pg. Nevertheless, high IgA1-Bp/IgA1-Pg > 1 or IgA1-E2/IgA1-Pg > 1 combined with low IgG2-E2 ≤ 4 + IgG2-Pg ≤ 2 were revealed in HW (27.7% and 28.8%) more frequently, than in BCP (19.7%, p = 0.06 and 17.9%, p = 0.008). Excess of IgA1-Bp and IgA1-E2 levels over IgA1-Pg in combination with high IgG2-E2 and IgG2-Pg levels in HW is associated with ER+/PR+ BC stage I condition and may serve as an marker for preventive BC therapy by the targeted ER modulators.

Russian Journal of Immunology. 2023;26(1):27-40
pages 27-40 views

Incidence of IgA antibodies specific to benzo[a]pyrene and steroid hormones in women with colorectal cancer and breast cancer

Averianov A.V., Antonov A.V., Zhivotovskiy A.S., Kostyanko M.V., Vafin I.A., Kolpinskiy G.I., Glushkov A.N.


Formation of DNA adducts of chemical carcinogens is a trigger for carcinogenesis. Adducts of benzo[a]pyrene metabolites and estradiol metabolites with DNA have been found in normal and tumor cells in healthy women and patients with breast and colorectal cancer. These low-weight compounds in macromolecular complexes induce the synthesis of specific antibodies. Previously, the presence of specific antibodies against benzo[a]pyrene (IgA-Bp), estradiol (IgA-Es) and progesterone (IgA-Pg) was revealed in breast cancer patients. The aim of this study is to identify the putative features of the IgA-Bp, IgA-Es, and IgA-Pg formation in postmenopausal women with colorectal cancer, in comparison with healthy and breast cancer patients. Using a noncompetitive enzyme-linked immunosorbent assay, the content of these antibodies was studied in the blood serum of healthy women (n = 401), patients with colorectal cancer (n = 219) and breast cancer (n = 1469) using conjugates of Bp, Es, and Pg with bovine serum albumin as adsorbed antigens. When compared with healthy people, the patients with colorectal cancer exhibited higher incidence of IgA-Bp > 3 (75% vs 37%, p < 0.0001, OR = 5.0), as well as more common levels of individual antibody ratios: IgA-Bp/IgA-Es > 1 (82% vs 41%, p < 0.0001, OR = 6.5); IgA-Bp/IgA-Pg > 1.5 (77% vs 20%, p < 0.0001, OR = 13.4); IgA-Es/IgA-Pg > 1 (89% vs 48%, p < 0.0001, OR = 8.7). In breast cancer patients, compared with healthy people, high IgA-Bp values ( > 3) were more common (45% vs 37%, p < 0.004, OR = 1.4), as well as increased IgA-Bp/IgA-Es ratio > 1 (57% vs 41%, p < 0.0001, OR = 1.9), IgA-Bp/IgA-Pg > 1.1 (71% vs 36%, p < 0.0001, OR = 4.4) and IgA-Es/IgA-Pg > 1.1 (71% vs 41%, p < 0.0001, OR = 3.5). In patients with colorectal cancer, compared with patients with breast cancer we have found higher incidence of increased IgA-Bp values ( > 3) (75% vs 45%, p < 0.0001), IgA-Es > 3 (53% vs 39%, p < 0, 0001), and of IgA-Pg > 2 (52% vs 44%, p = 0.025), as well as IgA-Bp/IgA- Es > 1 (82% vs 57%, p < 0.0001, OR = 50.8 ); IgA-Bp/IgA-Pg > 1.5 (77% vs 49%, p < 0.0001); IgA-Es/IgA-Pg > 1.1 (85% vs 71%, p < 0.0001). The apparently high serum IgA-Bp levels reflect the formation of DNA-Bp adducts at large scale in target cells in colorectal cancer compared with healthy women and breast cancer patients, due to direct exposure of colon epithelium to Bp from food. Immunoassay for IgA-Bp, IgA-Es and IgA-Pg is proposed for assessing individual risk of colorectal cancer in postmenopausal women. The ratios of IgA Bp/IgA-Pg levels > 1.5 represent the most informative marker of individual risk for colorectal cancer.

Russian Journal of Immunology. 2023;26(1):41-48
pages 41-48 views

Prognosis of immune state following basic therapy and thymalin treatment in patients with severe COVID-19 infection

Kuznik B.I., Smolyakov Y.N., Shapovalov K.G., Tereshkov P.P., Konnov V.A., Chalisova N.I.


Significant changes in cellular and humoral immunity are observed in new coronavirus infection (COVID-19). The “cytokine storm” develops in cases of severe clinical course, being accompanied by significantly increased levels of pro-inflammatory cytokines, often associated with suppression of immune response. At the same time, the prediction of the immune status is an urgent task, thus allowing timely correction of current therapy. The aim of our research was to evaluate predictive capability for the immune system changes on the 6th day of COVID-19 disease when using standard therapy, or with addition of immunocorrector thymalin to the treatment regimen. A retrospective study was conducted in 87 patients with severe COVID-19. All patients were divided into 2 groups, i.e., (1) controls who received basic treatment; (2) basic treatment supplied with thymalin (10 mg, intramuscular injections daily for 5 days). Assessment of severity and clinical course of COVID-19, and basic treatment regimen for the patients corresponded to current version of the interim Guidelines from the Ministry of Health of the Russian Federation “Prevention, diagnosis and treatment of a new coronavirus infection COVID-19”. Laboratory studies included complete blood counts, immunogram parameters with the calculation of the ratio of certain types of leukocytes were performed on the 1st and 6th days of observation. Statistical evaluation was made using scripts of the specialized statistical analysis language R (http://cran.r-project.org) version 4.1.3. The blood parameters were evaluated using the binary classification method. The changes in parameters of cellular immunity were classified by means of ROC-analysis.

We have found that the basic therapy of severely ill COVID-19 patients was not followed by recovery of immune status on the 6th day from the start of treatment. At marginal level, one can only suggest a probable prediction of increase in WBC and MON counts, a decrease in CD4+, NK and CD8+perNK, as well as the CD4+/CD8+ ratio. Addition of thymalin to the basic therapy is largely accompanied by the normalization of immunogram parameters. At the same time, it is possible to predict, with “excellent” rating, an increased number of T-LIM, including CD4+ and B-LIM, and, with “good” rating, an increase in the total numbers of LIM, as well as CD8+, HLA-CD3+DR+ and NK cells. The data obtained in severe cases of COVID-19 make it possible to predict changes in immune status, and, hence, the course of the disease, at a high degree of probability.

Russian Journal of Immunology. 2023;26(1):49-56
pages 49-56 views


Anticytokine activity of Candida spp. and their ability to produce cytokine-like substances

Pashinina O.A., Pashkova T.M., Kartashova O.L., Morozova N.V.


Bacteria are known both to exhibit anticytokine activity (ACA), i.e., to secrete extracellular compounds which inactivate / neutralize various cytokines, and to produce cytokine-like substances (CPV) released to the culture medium. At the same time, the ability of Candida to secrete such substances has not been previously studied. The purpose of our study was to determine the presence of ACA and the ability to produce CPV in Candida strains isolated from the reproductive tract of apparently healthy pregnant women.

The experimental series included 26 clinical strains of Candida spp. isolated from vaginal secretions. Isolation and identification of fungal species was based on morphological and biochemical criteria. ACA against IL-4, IL-6, IL-8, IL-10, IL-17A and TNFá were detected following 2-hour co-incubation of fungal suspensions with solutions of the distinct cytokines at a ratio of 1:1. Determination of the cytokine concentrations was carried out by ELISA using the Cytokin kits (St. Petersburg). ACA amounts were defined as the percentage of cytokine inactivation in experimental samples compared to the control expressed as pg/mL. Their ability to produce CPV was expressed as percentage of cytokine production in experimental specimens compared to the controls (pg/mL). The obtained data were subjected to statistical evaluation.

ACA of Candida spp. and their ability to produce CPV were revealed for the first time. with appropriate differences established between the studied Candida species, i.e., cultures of C. non-albicans showed ACA more often for the pro-inflammatory cytokines; C. albicans isolates showed more frequent production of the substances, similar to the pro-inflammatory cytokines IL-8, IL-17A, and TNFá. Expression of ACA against IL-10 and ability to produce the anti-inflammatory IL-4- and IL-10-like substances were significantly higher in the cultures of C. non-albicans species.

The results of these experiments expand our knowledge on the spectrum of Candida biological activities and require further study.

Russian Journal of Immunology. 2023;26(1):57-62
pages 57-62 views

PD-1 expression on T cell subsets from peripheral blood of patients with allergic diseases

Blinova E.A., Galdina V.A., Sukhova N.M., Demina D.V.


Pathogenesis of allergic diseases is based on the activation of Th2 immune response. T cell response to antigens is known to be regulated by various co-stimulatory and inhibitory signals; inhibitory receptors include PD-1 and Tim-3 molecules. Interaction of the PD-1 receptor with its ligands leads to suppression of activation, proliferation of T cells and production of cytokines by these cells. In chronic bacterial and viral infections, increased expression of PD-1 and Tim-3 on T cells is a marker of cellular “exhaustion”, since the cytokine production is reduced in cells positive for these inhibitory receptors. Involvement of the co-inhibitory PD-1 receptor into pathogenesis of allergic diseases has not been sufficiently studied, and the data available from the current literature are scarce and contradictory. Therefore, the aim of our study was to evaluate the expression level of co-inhibitory PD-1 and Tim-3 receptors on CD4+ and CD8+T cells, as well as expression of PD-1 on allergen-specific Th2A cells in allergic bronchial asthma (BA) and allergic rhinitis (AR) beyond the pollination season. We used the flow cytometry method to assess immune phenotype of peripheral blood cells from blood donors and patients with allergic diseases, in order to evaluate expression of PD-1 and Tim- 3 markers. The study included 7 patients with allergic asthma, 10 patients with AR beyond the exacerbation phase, and 14 healthy, allergy-free individuals. It was found that the number of CD4+ and CD8+ cells expressing the PD-1 molecule in peripheral blood of patients with BA and AR was within the ranges of healthy individuals. A decrease in CD4+ cells expressing Tim-3 marker was observed only in the group of patients with AR which may be caused both by the absence of pollen allergens and remission of the disease. The content of allergen-specific Th2A cells (CD4+CD45RO+CD27-CRTh2+CD161+) in patients with AR and BA was increased relative to the control group, thus suggesting a pathogenetic significance of this cell population for allergic diseases. However, the level of PD-1 expression on Th2A cells, as well as on the main T cell subpopulations, was comparable to donor values. This finding may suggest that PD-1 may be considered not only a marker of “exhaustion” as shown for chronic viral infections. Moreover, it also may reflect the activation status of various T cell subpopulations, including allergen-specific Th2A cells in allergic conditions.

Russian Journal of Immunology. 2023;26(1):63-68
pages 63-68 views

Issues of specific in vitro allergological diagnosis of atopic conditions

Barilo A.A., Smirnova S.V., Belenyuk V.D., Savchenko A.A., Borisov A.G.


There is a steady increase in the prevalence of allergic diseases of atopic origin worldwide, e.g., atopic bronchial asthma (ABA) and atopic dermatitis (AD). Identification of a causally significant allergen in allergic patients is crucial for the diagnosis, therapy and prevention of allergic diseases. Korea has developed the Allergy-Q® multiplex test to detect specific IgE. Allergy-Q® is based on an immunoblotting method using a nitrocellulose membrane as a solid phase for allergen immobilization and can detect allergen-specific IgE simultaneously to 107 allergens. Our aim was to conduct a comparative analysis for detectable allergen-specific IgE antibodies to food, fungal, pollen, household, epidermal allergens in blood serum by immunoblotting method using the Allergy-Q® test system in patients with atopic dermatitis, atopic bronchial asthma and psoriasis.

The study included patients with atopic dermatitis (AD, group 1, n = 9), atopic bronchial asthma (ABA, group 2, n = 14) and psoriasis (PS, group 3, n = 17). The concentration of total immunoglobulin E and allergen-specific immunoglobulins of class E in blood serum to 32 most common food, fungal, pollen, household, epidermal allergens was determined by the immunoblotting method using the Allergy-Q® test system (Korea).

We have found that sensitization of atopic origin was observed in all patients with AD (n = 9), in 85.7% (n = 12) of patients with atopic bronchial asthma, and in 47.1% (n = 8) of patients with psoriasis. Polyvalent sensitization was shown to prevail in all groups of the examined persons. When studying the spectrum of sensitization to food allergens, a significantly increased frequency of positive reactions to cow’s milk protein was found in the group of patients with AAA as compared with AD and PS groups. Among all studied groups, sensitization to the Alternaria fungi was found at the highest frequency in the group of patients with ABA. Sensitization to ragweed pollen was very common in all groups of patients. Sensitization to household and epidermal allergens in the groups with AD and AAA was noted for all studied allergens with the highest positivity rates for the feline epithelium and dog dander.

In the present study, the Allergy-Q® system showed an agreement with preliminary data from a specific allergological examinations. This relationship suggests a potential for usage of the Allergy-Q® immunoblotting method as a highly effective alternative to other in vitro tests for diagnosing atopy. An advantage of the Allergy-Q® Multiplex Serum Allergen-Specific IgE Detection Kit is a short processing time, small amount of blood sample, and broader clinical information on the causative allergens.

Russian Journal of Immunology. 2023;26(1):69-76
pages 69-76 views

Complex inflammation indexes in patients with post-COVID syndromeНа сегодняшний день в мире активно занимаются выделением новых маркеров, характеризующих тяжесть состояния после перенесенного COVID-19. Для более детального понимания того, какие изменения вызывает вирус SARS-CoV-2 в организме человека и с чем взаимосвязано длительное сохранение симптомов после перенесенной новой коронавирусной инфекции, необходимо оценить связь между степенью тяжести после заболевания и показателями клинического анализа крови. Цель работы – выделение прогностических маркеров по клиническому анализу крови, коррелирующих с тяжестью состояния пациентов после перенесенной новой коронавирусной инфекции. В исследование включены 372 пациента в постковидный период. Исходное течение заболевания оценивалось с использованием шкалы клинического прогрессирования ВОЗ. Разделение пациентов на группы осуществлялось по шкале функционального состояния после COVID-19 (PCFS). В анализе крови обследуемых были выделены критические значения лабораторных показателей, которые были проанализированы на предмет наличия взаимосвязей с тяжестью синдрома. Проведен качественный анализ типов реакции иммунной системы в программе «Протист». Установлены наиболее статистически выраженные изменения у пациентов группы 1. Коэффициенты NLR, LMR, SII при сравнении с группой пациентов 1, 2, 3 были ниже. Индекс PLR был ниже при сравнении с группой пациентов 0. Других статистических различий между группами не выявлено. Поэтому интерес вызывает исследования качественных показателей. В группах больных 0, 1, 2 в основном определяется ареактивность иммунитета (71-92%), а в группах пациентов 3 и 4 уже у половины обследуемых наблюдается активация врожденного иммунитета, активация адаптивного иммунитета и иммунодефицит (угнетение иммунитета). Пациенты группы 2 занимали промежуточное состояние. В постковидный период общие гематологические нарушения не являются выраженными и достаточными для постановки диагноза. В большей степени необходимо ориентироваться на комплексные качественные показатели. Как один из основных качественных параметров у пациентов в постковидный период имеет смысл использовать «Методику определения типа реакции иммунитета по развернутому анализу крови» При этом оценка типа иммунной реакции позволяет не только выявить пациентов с постковидным синдромом, но и провести отбор пациентов, нуждающихся в противовоспалительной и детоксикационной терапии.

Sadovskiy I.S., Kruglova O.S., Savchenko A.A., Sobko E.A., Kasparov E.V., Demko I.V., Borisov A.G.


Today, the world is actively engaged in the selection of new markers that correlate with severity of the condition after recovery from COVID-19. For a more detailed understanding of the changes caused by SARS-CoV-2 virus in the human body, and assessment of the factors correlating with long-term persistence of symptoms after a new coronavirus infection, one should evaluate the relationships between the severity of the disease and the indices of clinical and biochemical blood tests. The purpose of the work was to reveal prognostic markers based on clinical blood testing which correlate with severity of the patient’s condition after a new coronavirus infection.

The study included 372 patients in the post-COVID period. Initial course of the disease was assessed using the WHO Clinical Progression Scale. The patients were divided into groups according to the Post-COVID-19 Functional Status Scale (PCFS). When performing clinical blood analysis of subjects, critical points of laboratory parameters were identified and analyzed for presence of relationships with severity of the syndrome. Qualitative analysis of distinct types of immune reactions was carried out using the Protist software.

The more pronounced statistically significant changes were found for the group 1. The NLR, LMR, SII coefficients were lower when compared with group 1, 2, 3. The PLR index was lower when compared with group 0. There were no other statistical differences between the groups. Therefore, the study of qualitative indexes is of interest. Suppressed immune respose (71-92%) was revealed, mainly, in groups 0, 1, 2. Activation of innate immunity, increased adaptive immunity and immunodeficiency (immune suppression) in groups 3 and 4 are observed in a half of the cases. The patients from group 2 took an intermediate position.

Over the post-COVID period, general hematological disorders are not pronounced and sufficient for making a diagnosis. Therefore, one should bring more attention to complex qualitative indicators. It makes sense to use the “Methods for determining the type of immune response by a comprehensive blood test” as one of the main qualitative parameters in patients in the post-COVID period. At the same time, assessing the type of immune response allows not only to identify patients with post-COVID syndrome, but also to select patients who require anti-inflammatory and detoxication therapy.

Russian Journal of Immunology. 2023;26(1):77-86
pages 77-86 views


Autoinflammatory undifferentiated syndrome

Tolstykh A.V., Popova L.Y., Albakasova A.A., Usenkova N.N.


Identification of primary immunodeficiencies (PID), distinction of their nosological forms and timely admoinistered therapy for this disorders frepresent topical problems of modern immunology. According to the PID registry of the National Association of Experts in the Field of Primary Immunodeficiencies (NAEPID), as of 2021, 3617 cases of this disease were diagnosed in Russian Federation (RF). The prevalence of PID in Russian Federation is 2.48 per 100,000 population. Currently, autoinflammatory syndromes (AIS) comprise rare, genetically determined disorders. According to the NAEPID registry data, of the PID register, 541 cases of autoinflammatory syndrome (AIS) were registered in the Russian Federation (2021). Timely diagnosis of AIS is especially important in young children who have similar phenotypic signs, in order to reduce the number of deaths and prevent disability. According to the PID registry, the median diagnostic delay in Russia is 27 months. The purpose of this work is to update information about the autoinflammatory syndrome that clinicians may encounter, e.g., pediatricians, rheumatologists, hematologists and other specialists. This syndrome requires a complex differential diagnostic algorithm for clinicians and is often subject to multidisciplinary approach, involving specialists of different profile. This article describes a clinical case of a 3-year-old child S. with a diagnosis of Primary immunodeficiency: autoinflammatory syndrome, undifferentiated. The patient was diagnosed since the age of 5 months, when periodic rises in body temperature to febrile values were registered once a month. Later on, the fever episodes were observed 2 times a month. The diagnosis was made at the place of residence as secondary immunodeficiency virus-associated state (CMV infection). CMV viremia was canceled against the background of ongoing treatment, but the inflammatory attacks persisted. Molecular genetic studies did not reveal any defects. In view of poor response to NSAID therapy and prednisone prescribed at a dose of 1-1.5 mg/kg/day, he was admitted to the Dmitry Rogachev Research Medical Cemter. The child was finally diagnosed with PID, and therapy was initiated with a selective competitive inhibitor of TNFa – etanercept at a dose of 0.8 mg/kg/day once a week. Hence, the autoinflammatory syndrome in children is difficult to diagnose and select therapy, and it may be unfavorable prognostically.

Russian Journal of Immunology. 2023;26(1):87-94
pages 87-94 views

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