IgA Deficiency Research Articles

Demographic, clinical, immunological, and molecular features of iranian national cohort of patients with defect in DCLRE1C gene

BackgroundDCLRE1C gene mutation leads to Artemis deficiency, a severe form of combined immunodeficiency (SCID). Impaired DNA repair and block in early adaptive immunity maturation results in T-B-NK+ immunodeficiency associated with radiosensitivity. Recurrent infections early in life are the main characteristic of Artemis patients.MethodAmong 5373 registered patients, 9 Iranian patients (33.3% female) with confirmed DCLRE1C mutation were identified since 1999–2022. The demographic, clinical, immunological and genetic features were collected through retrospective investigation of medical records and using next generation sequencing.ResultsSeven patients were born in a consanguineous family (77.8%). The median age of onset was 6.0 (5.0–17.0) months. Severe combined immunodeficiency (SCID) was clinically detected at a median (IQR) age of 7.0 (6.0–20.5) months, following a median diagnostic delay of 2.0 (1.0–3.5) months The most typical first presentation was pneumonia (44.4%) and otitis media (3.33%), followed by BCG lymphadenitis (22.2%) and gastroenteritis (11.1%). The most prevalent manifestations were respiratory tract infections (including otitis media) (66.6%) and chronic diarrhea (66.6%). In addition, juvenile idiopathic arthritis (P5) and celiac disease and idiopathic thrombocytopenic purpura (P9) as autoimmune disorders were reported in 2 patients. All patients had reduced B CD19+ and CD4+ cell counts. IgA deficiency occurred in 77.8% of individuals.ConclusionRecurrent infections particulary respiratory tract infection and chronic diarrhea during the first months of life in patients born to consanguineous parents should raise the suspicion for inborn errors of immunity, even in the presence of normal growth and development.

Racial and ethnic differences in patients enrolled in the national bronchiectasis and nontuberculous mycobacteria research registry

Demographic and socioeconomic factors are recognized to contribute to disparities in healthcare outcomes. Originally, bronchiectasis was described in a population of predominantly White ethnic group of patients in which racial disparity could not be identified. The U.S. Bronchiectasis Research Registry (BRR), a centralized database of adult patients with bronchiectasis and/or NTM from 18 clinical institutions across the U.S., was created to support the research of this condition. The aim of this study is to describe the racial and ethnic distribution of patients enrolled in the BRR and evaluate factors associated with healthcare disparities within manifestations of and/or the care delivered to this population.At the time of this study, 3600 patients with bronchiectasis and/or NTM were enrolled in the BRR. Of those, 3510 participants were included in these analyses. The population was predominantly non-HispanicWhite (n = 3143, 89.5%), followed by Hispanic or Latino (n = 149, 4.3%), Asian (n = 130, 3.7%) and non-Hispanic Black (n = 88, 2.5%) participants. Testing for cystic fibrosis, immunoglobulin deficiency, and mycobacteria was not different between races, but non-Hispanic Black patients were tested less frequently for alpha-1 antitrypsin (A1AT) deficiency compared to other groups (P = 0.01). The four groups did not differ in the proportion of Pseudomonas aeruginosa or Hemophilus influenzae. There was no statistically significant difference in use of high-frequency chest wall oscillation, pulmonary rehabilitation services, or suppressive macrolide treatment across the groups (P > 0.05).There is a disproportionately high percentage of non-Hispainc White patients compared to non-Hispanic Black patients and Hispanic or Latino patients in the BRR. However, we found an overall similarity of care of BRR patients, regardless of racial and ethnic group.

IgA Nephropathy: Pleiotropic impact of Epstein-Barr virus infection on immunopathogenesis and racial incidence of the disease.

IgA nephropathy (IgAN) is an autoimmune disease in which poorly galactosylated IgA1 is the antigen recognized by naturally occurring anti-glycan antibodies, leading to formation of nephritogenic circulating immune complexes. Incidence of IgAN displays geographical and racial disparity: common in Europe, North America, Australia, and east Asia, uncommon in African Americans, many Asian and South American countries, Australian Aborigines, and rare in central Africa. In analyses of sera and cells from White IgAN patients, healthy controls, and African Americans, IgAN patients exhibited substantial enrichment for IgA-expressing B cells infected with Epstein-Barr virus (EBV), leading to enhanced production of poorly galactosylated IgA1. Disparities in incidence of IgAN may reflect a previously disregarded difference in the maturation of the IgA system as related to the timing of EBV infection. Compared with populations with higher incidences of IgAN, African Americans, African Blacks, and Australian Aborigines are more frequently infected with EBV during the first 1-2 years of life at the time of naturally occurring IgA deficiency when IgA cells are less numerous than in late childhood or adolescence. Therefore, in very young children EBV enters "non-IgA" cells. Ensuing immune responses prevent infection of IgA B cells during later exposure to EBV at older ages. Our data implicate EBV-infected cells as the source of poorly galactosylated IgA1 in circulating immune complexes and glomerular deposits in patients with IgAN. Thus, temporal differences in EBV primo-infection as related to naturally delayed maturation of the IgA system may contribute to geographic and racial variations in incidence of IgAN.

Long-Term Immunological Memory of SARS-CoV-2 Is Present in Patients with Primary Antibody Deficiencies for up to a Year after Vaccination.

Some studies have found increased coronavirus disease-19 (COVID-19)-related morbidity and mortality in patients with primary antibody deficiencies. Immunization against COVID-19 may, therefore, be particularly important in these patients. However, the durability of the immune response remains unclear in such patients. In this study, we evaluated the cellular and humoral response to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antigens in a cross-sectional study of 32 patients with primary antibody deficiency (n = 17 with common variable immunodeficiency (CVID) and n = 15 with selective IgA deficiency) and 15 healthy controls. Serological and cellular responses were determined using enzyme-linked immunosorbent assay and interferon-gamma release assays. The subsets of B and T lymphocytes were measured using flow cytometry. Of the 32 patients, 28 had completed the vaccination regimen with a median time after vaccination of 173 days (IQR = 142): 27 patients showed a positive spike-peptide-specific antibody response, and 26 patients showed a positive spike-peptide-specific T-cell response. The median level of antibody response in CVID patients (5.47 ratio (IQR = 4.08)) was lower compared to healthy controls (9.43 ratio (IQR = 2.13)). No difference in anti-spike T-cell response was found between the groups. The results of this study indicate that markers of the sustained SARS-CoV-2 spike-specific immune response are detectable several months after vaccination in patients with primary antibody deficiencies comparable to controls.

IgE Deficiency as a Predictive Marker of Hypogammaglobulinemia

The significance of isolated IgE deficiency (IgE<2 IU/mL) is unclear, but we have hypothesized that low IgE is a sentinel marker for developing humoral immunodeficiency. Here we related total IgG levels at two points in time with IgE status in a large cohort of military personnel.

Assessment of low immunoglobulin levels and clinical manifestations in patients with mastocytosis.

Patients with a low IgG level alone or with low IgA or IgM levels have been reported to be susceptible to respiratory tract infections and recurrent sinusitis. Patients diagnosed with CVID have a higher prevalence of autoimmune diseases and lymphoid malignancies. Mastocytosis is a myeloproliferative disease, not typically associated with autoimmune disease or frequent infections. We sought to determine the distribution of immunoglobulins in children and adults with mastocytosis. Evaluate the impact of low immunoglobulins on the clinical management of patients with mastocytosis. We performed a 10-year retrospective analysis on 320 adult and pediatric patients with mastocytosis for immunoglobulins using an electronic medical query. We identified 25 adults and 9 children with one or more low immunoglobulins. Patient records were examined for a history of infections and autoimmune disorders. Serum immunoglobulins in children and adults with mastocytosis fell within a normal range. Among patients with low IgG levels alone or with low IgM and /or IgA, 20% had a history of infections and 20% of adults had autoimmune disorders. The most common infection was recurrent otitis media (OM). Patients with mastocytosis typically have normal immunoglobulins. With few exceptions, those with low immunoglobulins did not have frequent infections or autoimmune diseases. This data supports the conclusion that routine determination of immunoglobulins in patients with mastocytosis is not required and reserved for patients with clinical conditions, which might be related to an immunoglobulin deficiency.

Evaluation of Chronic Cough Etiologies in Children

Objective: Cough is one of the leading causes of hospitalization in children worldwide. It should be considered important because it can be a symptom of various serious diseases and affects the quality of life of the child. A differential diagnosis should be performed on all children with a chronic cough. This study aimed to determine the etiologies in patients evaluated for chronic cough. Material and Methods: The study included patients admitted to the pediatric immunology and allergy outpatient clinic, with the complaint of chronic cough between the ages of 0–18 years. Clinical and demographic characteristics of patients were recorded. Results: This study included 323 patients between the ages of 0-18years. The median age of the patients was 7 (interquartile range: 5-9.7) years. One hundred and forty five (45%) of the patients were female. One hundred seventy-nine (55.4%) patients had a family history of atopic diseases such as asthma and/or allergic rhinitis. The presence of aeroallergen sensitivity was demonstrated in 127 patients. One hundred and forty-four (44.6%) patients were diagnosed with asthma, 75 (23.2%) patients with wheezing, 54 (16.7%) patients with post-infectious cough, 43 (13.3%) patients with postnasal drip syndrome, 4 (1.2%) patients with gastroesophageal reflux, 2 (0.6%) patients with foreign body aspiration, and 1 (0.3%) patient with psychogenic cough. Moreover, two patients with asthma, and one patient with wheezing had reflux symptoms in addition to the diagnosis of asthma and wheezing. One patient was found to have a partial IgA deficiency, and 18 patients had hypogammaglobulinemia. Immunoglobulin replacement therapy was initiated for a patient with hypogammaglobulinemia. Of the 219 patients diagnosed with asthma or wheezing, 144 had an atopic disease in the family (p&amp;lt;0.001). It was found that one hundred and eighty-five patients (57.3%) had domestic smoke exposure. Furthermore, smoke exposure was observed in 58 (77.3%) of 75 patients under the age of 6 years who were followed up with the diagnosis of wheezing (p= 0.010). Conclusion: In our study, asthma, which is one of the most common causes of chronic cough in the literature, was found to be the most common cause. For a correct approach when making a differential diagnosis in pediatric patients presenting with chronic cough, the patient’s history, physical examination, laboratory tests, and risk factors should be evaluated as a whole with systematic evaluation.

Endoscopic sinus surgery outcomes in patients with chronic rhinosinusitis and immunoglobulin deficiencies

BackgroundPatients with chronic rhinosinusitis (CRS) and immunoglobulin deficiencies (ID) have more recalcitrant sinonasal disease and a subset of these patients undergo surgical management for their CRS. However, there is a paucity of literature on the surgical outcomes in this patient population and appropriate treatment algorithms for CRS in patients with ID. The objective of this study was to better elucidate the outcomes of endoscopic sinus surgery (ESS) in patients with ID in terms of disease-specific quality-of-life scores and the need for revision surgery.MethodsA case–control study was performed comparing adult patients with ID and healthy controls that had undergone ESS for CRS. Patients were matched based on age, sex, CRS phenotype, and preoperative Lund-Mackay score. The revision surgery rates, time to revision surgery, and changes in sinonasal outcome tests (SNOT-22) were evaluated.ResultsThirteen patients with CRS and ID were matched to 26 control patients with CRS. The revision surgery rate for cases and controls was 31% and 12%, respectively, but there was no statistical difference (p > 0.05). There was a clinically meaningful reduction in SNOT-22 scores in both groups from the preoperative to postoperative period [mean of 12 points in patients with ID (p = 0.323) and 25 points in controls (p < 0.001)], however, there was again no significant difference between cases and controls (p > 0.05).ConclusionOur data suggests that patients with ID have clinically meaningful improvement in SNOT-22 scores after ESS but may have higher revision rates than immunocompetent patients with CRS. ID are rare disease entities, thus most attempts at studying this cohort would be limited by sample size. Further homogenous data on immunoglobulin deficient patients is required for future meta-analysis to better understand the impact of ESS in patients with ID.

The role of serology in the diagnosis of coeliac disease.

Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the identification of unsuspected patients in at-risk CD groups, thereby increasing the number of CD diagnoses compared to the pre-screening era. Several markers for CD with a progressive diagnostic accuracy have been identified over the years, but only three of them, i.e. anti-tissue transglutaminase (anti-tTG), anti-endomysial (EmA) and anti-deamidated gliadin antibodies (DGP) are currently assessed in the daily clinical practice. A thorough review of the literature identified 44 original studies published between 1998 to 2022 for a total of 5098 pediatric and adult CD patients (without selective IgA deficiency) and 11930 disease controls. The results highlighted that anti-tTG IgA exhibited a higher sensitivity for CD (93.4%) than EmA IgA (92.8%), DGP IgG (81.8%) and DGP IgA (83.8%). The specificity of EmA IgA (99%) resulted to be higher than those of anti-tTG IgA (95.8%), DGP IgG (96.4%) and DGP IgA (92.1%). In patients with selective IgA deficiency, a condition closely related to CD, serological screening should include one of the three antibodies of IgG class, since anti-tTG, DGP and EmA have a very similar diagnostic accuracy in this clinical setting. According to age, there are two main diagnostic strategies for CD detection. In children, the revised ESPGHAN 2020 guidelines established that CD could be diagnosed in both symptomatic and asymptomatic children by high anti-tTG IgA titers (>10 times the cut-off) and EmA positivity with no need to obtain duodenal biopsy and HLA typing. In adult patients, although high tTG IgA titers (confirmed by EmA IgA positivity) correlate with villous atrophy, an intestinal biopsy is still considered mandatory for confirming CD diagnosis. Currently, a case finding approach in at-risk groups is preferred to mass screening for CD detection.

Diagnostic significance of immunophenotyping of peripheral blood lymphocytes in pediatric patients from the Autonomous Province of Vojvodina, Republic of Serbia

Introduction/Objective. Although lymphocyte immunophenotyping based on flow cytometry is a powerful tool in the diagnosis of many primary immunodeficiences (PID), there has been an increasing awareness of associated costs and the need for its reassessment as a screening tool. We present the results and diagnostic impact of immunophenotyping performed by flow cytometry in the University Children?s Hospital, Belgrade, in a series of patients referred from the Institute for Child and Youth Health Care of Vojvodina from July 2008 to July 2018. Methods. We reviewed the laboratory reports on numbers of B lymphocytes (CD19+), T lymphocytes (CD3+), natural killer cells (CD3?CD16/CD56+) and activated T cells (CD3+HLA-DR+), as well as CD4+ and CD8+ T cells in 198 children. Results. Patients were grouped by stated indication into the following eight categories: hypogammaglobulinemia (34), selective IgA deficiency and/or IgG subclass deficiency (43), various infections with no immunoglobulin deficiencies (67), asthma and/or allergies with no immunoglobulin deficiencies or infections (23), known or suspected autoimmune disorders (24), and miscellaneous diagnoses not accompanied by infections (7). In total, 159 (80.3%) findings were either completely within the respective reference range or exhibited only minimal aberrations. Four patients were diagnosed with Bruton?s disease and one with Artemis immunodeficiency. Nineteen patients were given immunoglobulin substitution to control infections and/or maintain immunoglobulin G levels. Conclusion. Lymphocyte immunophenotyping aids the diagnosis of PID in selected patients. We venture some thoughts on how the usefulness of this laboratory method could be improved in real-life tertiary care pediatric hospital settings.

Acute management of pneumonia in adult patients.

Acute management of pneumonia in adult patients.

Celijačna bolest kod dece

Celiac disease is a multisystemic autoimmune disease induced by gluten in wheat, rye, and barley. It is characterized by polygenic predisposition , high prevalence in members of the Caucasian race (1%), especially in close relatives (5-15%), very heterogeneous expression, and frequent association with other autoimmune diseases (3-10%), as well as selective deficiency of IgA and Down, Turner, and Williams syndromes. The basis of the disease and the key finding in its diagnostics is symptomatic or asymptomatic inflammation of the small intestinal mucosa, which is resolved by a gluten-free diet. Accordingly, the basis of the treatment involves an elimination diet, so the disorder itself, if timely recognized and adequately treated, is characterized by an excellent prognosis.

Assessment of risk factors in pyogenic liver abscesses in children.

Pyogenic liver abscess (LA) is a significant contributor to morbidity and mortality in developing countries like India. The risk factors predisposing to the LA specifically in children are not known. Studies done in the past largely remain inconclusive and have identified only probable causes. The cause of LA in children with no coexisting illness remains unknown. This prospective observational study was conducted at a tertiary teaching hospital located in New Delhi, India. All children between 2 months and 12 years of age with sonographically confirmed LA presenting to the hospital were included and managed with appropriate intravenous antibiotics and relevant investigations. A total of 52 children were included. The mean age was 6 years and 4 months, and the male: female ratio was 1.4:1. Around 50% of the patients were malnourished. Fever, abdominal pain and loss of appetite were the most common symptoms. Nine patients (17%) were managed conservatively, 13 (25%) needed percutaneous needle aspiration and 30 (57.69%) required drainage using a pigtail catheter. Poor socioeconomic status and anaemia were found to be the most commonly associated risk factors. Selective immunoglobulin A (IgA) deficiency was the most common primary immunodeficiency disorder followed by T-cell defect. On multivariate analysis, it was seen that in those with clinical icterus, gamma-glutamyl transferases >350 IU/m, and those with impending rupture, the time to defervescence was significantly different (P = 0.05). Poor socioeconomic status causing malnutrition emerged as a significant risk factor for LA in children. Selective IgA deficiency was the most common immunodeficiency seen in a few children. Adopting a conservative approach like aspiration and percutaneous drainage led to lower mortality and good recovery rates.

Selective immunoglobulin A deficiency in a sample of Iraqi blood donors in the National Blood Transfusion Center-Baghdad

BACKGROUND: Selective immunoglobulin (Ig) A deficiency is the most common Ig deficiency in the world. Serum IgA level is reduced, 7 mg/dL (70 μg/mL), thus, associating with a wide range of clinical symptoms and signs that are related directly and indirectly to disturbances in the body immune system. Selective IgA deficiency patients are known to develop antibodies against lacking IgA. Whenever these antibodies are found in an individual's circulating blood, a severe allergic response and anaphylaxis can ensue upon receiving IgA containing blood or blood components. Hence, certain protocols are recommended in populations with increased prevalence of selective IgA deficiency including the use of red blood cell (RBC) washing machines or IgA-deficient blood components. Both measures are costly and require special maintenance procedures.objectives: The aim of this study was to screen for the presence of selective IgA deficiency in a group of blood donors and its probable role in the events of transfusion reactions that frequently occur which may necessitate the application of protocols that involve sophisticated procedures and highly-demanding machines.SUBJECTS AND METHODS: This was a laboratory study that included 300 volunteer blood donors in the National Blood Transfusion Center. They were screened for their serum IgA levels. Related questionnaire was conducted and chemiluminescence immunoassay technique with MAGLUMI 600 series machine was used. This machine is already used by the National Center of Teaching Laboratories for variety of biochemical and immunological assays. Serum IgA levels then were plotted against age, blood group and smoking status.RESULTS: No single case of Selective IgA deficiency was documented. Serum IgA levels showed increased levels with age and smoking demonstrated no significant effect on the Ig levels.CONCLUSION: In light of these results, it does not seem urgent or necessary at this point of time to introduce the highly demanding and expensive RBC washing machines in blood banks in Iraq. We recommend conducting a larger study that is more comprehensive with wider involvement of population, considering age, gender, and ethnicity.

The spectrum of inborn errors of immunity: a single tertiary center retrospective study in Alborz, Iran.

Background. Inborn errors of immunity (IEIs) are a group of heterogeneous disorders with inherited faults in the immune system that increase susceptibility to infections, malignancies, lymphoproliferation, and autoimmune/autoinflammatory disorders. Methods. We retrospectively studied the demographic characteristics, clinical features, and immunological profiles of the 90 IEIs patients, who were diagnosed and classified according to the European Society for Immunodeficiencies (ESID) and International Union of Immunological Societies (IUIS) criteria from July 2010 to June 2021. The study was carried out in the Non-communicable Diseases Research Center, Imam Ali Hospital, Alborz, Iran. Results. Within a period of 11 years, 53 (58.9%) males and 37 (41.1%) females were diagnosed and followed up for 20 IEI disorders. The median (IQR) age of onset, age of clinical diagnosis and diagnostic delay was 0.7 (0.08-2.0), 3.18 (1.0-8.0) and 1.5 (0.17-5.0) years, respectively. Twelve patients (36.4%) had a positive family history of IEI, and the majority of patients (84.5%) had recurrent infections. Pneumonia (51.7%) was the most common clinical manifestation among IEI patients, followed by skin complications (46.2%). The most frequently diagnosed IEI was immunoglobulin A deficiency (IgAD) (14.4%) and severe combined immunodeficiency (SCID) (11.1%). Predominantly antibody deficiencies group (36.7%) was the most common category, followed by combined immunodeficiencies with associated or syndromic features group (27.8%). Conclusions. IEIs have different patterns within populations with high consanguinity. There is a need to search for underlying genetic and epigenetic factors in most common IEIs in Alborz.

Celiac disease - a comprehensive review

Celiac disease is a multisystemic autoimmune disease induced by gluten in wheat, rye, and barley. It is characterized by polygenic predisposition, prevailing prevalence in members of the white population (1%), especially in close relatives (5?15%), very heterogeneous expression and frequent association with other autoimmune diseases (3?10%), as well as selective deficiency of IgA and Down, Turner, and Williams syndromes. The basis of the disease and the key finding in its diagnostics is gluten-sensitive enteropathy, i.e., non-specific inflammation of the small intestinal mucosa which resolves by gluten-free diet. In addition to enteropathy, whether symptomatic or asymptomatic, the disease is also characterized by various extraintestinal manifestations, and even very serious complications. Therapy is based on a lifelong glutenfree diet, so that the disorder, if diagnosed in time and treated consistently, has an excellent prognosis.

Recurrent Parotitis in Monozygotic Twins with Pseudohypoaldosteronism and IgA Deficiency

Recurrent Parotitis in Monozygotic Twins with Pseudohypoaldosteronism and IgA Deficiency

Clinical profile of patients with seronegative celiac disease.

This study aimed to determine the clinical profile of patients with seronegative celiac disease (SNCD). Celiac disease (CD) is mainly diagnosed based on positive serology and duodenal mucosal atrophy, but some patients have negative serology. Their diagnosis has some limitations; delays in diagnosis are likely accompanied by a poor prognosis and a high risk of developing complications of CD. In this retrospective study, 1115 patients were evaluated for CD with mucosal atrophy between 2010 to 2020. SNCD diagnosis requires genetic CD predisposition and improvement of both clinical symptoms and regrowth of duodenal villi after 12 months of a gluten-free diet (GFD) for all patients with IgA deficiency, other IgG-based serology for diagnosis of celiac was done and if these antibodies were negative, consider them as possible SNCD. If they had positive DQ2-DQ8 and improvement of clinical symptoms and mucosal atrophy after 12 months of GFD were confirmed SNCD. Of the 1115 study subjects, 27 had SNCD, 1088 had SPCD with a mean age of 29.7±15.7 years (1 to 76 years) in seropositive celiac disease (SPCD) subjects and 37.1±16.3 years (6 to 63 years) in SNCD participants and 19 female patients with SNCD were presented. The BMI of SNCD and SPCD patients were reported 23.9 and 21.4, respectively. In addition, SPCD subjects were more likely but not statistically significant to have a positive family history. Villous atrophy was shown in 100% SNCD and 95.6% SPCD cases. Scalloping and fissuring in duodenal biopsies were reported in 60% of SNCD and 84.5% of SPCD patients. There was some other cause of seronegative villous atrophy including 3 patients with Crohns disease, 2 with common variable immunodeficiency, 2 drug and one patient with peptic duodenitis. Anemia, neurological symptoms, and liver function tests (LFT) abnormality were common extra intestinal manifestations in SNCD individuals. Levels of Thyroid peroxidase (TPO), TSH were measured, it had been detected that SNCD cases had a higher rate of co-occurrence with thyroid diseases also SPCD cases showed a higher rate of co-occurrence with diabetes. Among patients with celiac disease 2.4% are SNCD. SNCD are older than SPCD at the time of diagnosis and have higher BMI. Most common of cause of seronegative enteropathy also is SNCD followed by inflammatory bowel disease (IBD) common variable immunodeficiency (CVID), medication use, and duodenitis, in this area.

Evaluation of humoral immune deficiency in Indian patients with bilateral bronchiectasis with no apparent aetiology.

Infections continue to be the leading aetiology of bronchiectasis in developing countries like India. Among non-infectious cases, the majority will have no identifiable cause despite extensive evaluation. Recently, immunodeficiency has been recognized as an important aetiology, but data on its prevalence remain rather sparse. The objective of this study is to evaluate the prevalence of humoral immunodeficiency in a cohort of adults with bilateral bronchiectasis with no apparent aetiology. This is the single-site study from Christian Medical College (Vellore, India) of adults with HRCT-proven non-infectious bronchiectasis. Humoral immunity was assessed through quantitative analysis of immunoglobulins and IgG subclass levels. Among 158 cases, immunoglobulin deficiency was found in 15%. Low IgM was the most predominate finding (7%), followed by common variable immunodeficiency (3%) and low IgA (2.5%). In addition, IgG subclass deficiency was found in 5%. In 53% of cases, no specific aetiology could be identified. Humoral immune deficiency is present in a significant proportion of patients with non-infectious bronchiectasis. Routine measurement of serum immunoglobulins should therefore be considered as part of the evaluation.

The reasons for the formation of tolerance to food antigens

One of the main issues of the peculiarities of the immune reactions of the gastrointestinal tract is the mechanisms of ensuring tolerance to food antigens. Concentrations of antibodies to food antigens actually reflect the state of the intestinal mucosa barrier function, and the degree of penetration of antigens into the blood determines the level of immune response to them. The aim of the study was to determine the risk criteria for violation of tolerance to food antigens. Material and methods. The study included the results of a survey and examination of 1334 adults living in the north of the European part of the Russian Federation, including 1100 born in the North, of which 970 were women and 364 were men. The average age of the respondents was 45.5±1.0 years. The comparison group consisted of 344 patients with pathology of the gastrointestinal tract who applied to the medical company "Biocor". The content of immunoglobulins (Ig) G to food antigens, total IgA, cytokines (tumor necrosis factor α, interleukin-6, interleukin-4) in blood serum were determined by enzyme immunoassay. Results. Rural residents often (more than 28%) have elevated concentrations of IgG to potato, river fish, wheat and rye antigens. Urban residents have the most pronounced decrease in tolerance to food antigens of chicken, cod, beef and pork. In healthy individuals, elevated (>100 ME/ml) concentrations of antibodies to meat products are recorded in the range of 11.3-13.9%, to dairy antigens - 11.5-14.1%, cereals - 11.9-13.4%. Slightly less frequently, elevated concentrations of antibodies to fish antigens (7.5-10.1%), vegetables (3.8-7.0%) and fruits (4.9-6.5%) are detected. In inflammatory and oncological diseases of the gastrointestinal tract, the content of antibodies to food antigens increases sharply. On average, the frequency of impaired tolerance to food antigens in patients is 2.7-6.1 times higher than in healthy individuals. Conclusion. Violation of tolerance to food antigens is associated with an increase in blood pro-inflammatory cytokines, mainly interleukin-6. In practically healthy individuals, a decrease in tolerance to food antigens is associated with a deficiency of blood IgA. The risk criteria for violation of the diet or consumption of low-quality foods may be an increase in the frequency of detection of elevated concentrations of antibodies to meat products in 14.6±3.0%, fish - 10.7±2.3%, cereals - 13.7±1.6%, dairy products - 14.8±1.5%, vegetables - 7.8±2.4% and fruits - 6.9±5.8%.