Селективный дефицит иммуноглобулина A в практике педиатра

Дмитриева Ю.А., Захарова И.Н., Радченко Е.Р. Селективный дефицит иммуноглобулина A в практике педиатра. Педиатрия. Consilium Medicum. 2019; 3: 122–126. DOI: 10.26442/26586630.2019.3.190633

________________________________________________

Dmitrieva Iu.A., Zakharova I.N., Radchenko E.R. Selective IgA deficiency in pediatrician practice. Pediatrics. Consilium Medicum. 2019; 3: 122–126.
DOI: 10.26442/26586630.2019.3.190633

Читать PDF

Аннотация

Селективный дефицит иммуноглобулина (Ig) А является наиболее распространенным первичным иммунодефицитным состоянием. В большинстве случаев заболевание протекает бессимптомно, у остальных больных клинические проявления могут включать в себя рецидивирующие инфекционные, аллергические и аутоиммунные заболевания. Раннее выявление селективного дефицита IgA может определить патогенетическое обоснование частого возникновения инфекционных и рефрактерного течения атопических заболеваний, способствовать своевременной корректной диагностике ассоциированных с первичным иммунодефицитным состоянием патологий, а также предотвратить развитие тяжелых анафилактических реакций на переливание препаратов крови. В статье представлены данные о структуре и функциях IgA в организме, определены патогенетические механизмы развития его дефицита, проанализированы клинические проявления заболевания. Обзор литературных данных дополнен обсуждением двух клинических случаев, демонстрирующих значение своевременной диагностики дефицита IgA.
Ключевые слова: дети, первичные иммунодефицитные состояния, иммуноглобулин А, селективный дефицит иммуноглобулина А, атопические заболевания, респираторные инфекции, кишечные инфекции, аутоиммунная патология, целиакия, микробиота.

________________________________________________

Selective IgA deficiency is the most common primary immunodeficiency disorder. Most patients with IgA deficiency are asymptomatic, other patients can develop recurrent infectious, allergic and autoimmune diseases. Early detection of selective IgA deficiency can determine the pathogenetic reasoning of frequent infectious and refractory course of atopic diseases, contribute to the timely correct diagnosis of disorders associated with the primary immunodeficiency, and also prevent the development of severe anaphylactic reactions to blood transfusion. The article provides data on IgA structure and functions, determines the pathogenetic mechanisms of its deficiency, and analyzes the clinical manifestations of the disease. A literature review is complemented by a discussion of two clinical cases demonstrating the importance of timely diagnosis of IgA deficiency.
Key words: children, primary immunodeficiency disorders, immunoglobulin A, selective IgA deficiency, atopic diseases, respiratory infections, intestinal infections, autoimmune pathology, celiac disease, microbiota.

Список литературы

1. Geha RS, Notarangelo LD, Casanova JL et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol 2007; 120: 776–94.
2. Yel L. Selective IgA Deficiency. J Clin Immunol 2010; 30 (1): 10–6.
3. Latiff AH, Kerr MA. The clinical significance of immunoglobulin A deficiency. Ann Clin Biochem 2007; 44 (Pt 2): 131–9.
4. Cunningham-Rundles C. Physiology of IgA and IgA deficiency. J Clin Immunol 2001; 21: 303–9.
5. Woof JM, Kerr MA. The function of immunoglobulin A in immunity. J Pathol 2006; 208: 270–82.
6. Russell MW, Sibley DA, Nikolova EB et al. IgA antibody as a non-inflammatory regulator of immunity. Biochem Soc Trans 1997; 25: 466–70.
7. Corthésy B. Roundtrip ticket for secretory IgA: role in mucosal homeostasis? J Immunol 2007; 178: 27–32.
8. Дранник Г.Н. Клиническая иммунология и аллергология. М.: Медицинское информационное агентство, 2003.
[Drannik G.N. Clinical immunology and allergology. Moscow: Medical News Agency, 2003 (in Russian).]
9. Cerutti A, Rescigno M. The biology of intestinal immunoglobulin A responses. Immunity 2008; 28: 740–50.
10. Bäckhed F, Ley RE, Sonnenburg JL et al. Host-bacterial mutualism in the human intestine. Science 2005; 307: 1915–20.
11. Macpherson AJ, Geuking MB, McCoy KD. Immune responses that adapt the intestinal mucosa to commensal intestinal bacteria. Immunology 2005; 115: 1531–62.
12. Corthésy B. Multi-faceted functions of secretory IgA at mucosal surfaces. Front Immunol 2013; 4: 185.
13. Iankov ID, Petrov DP, Mladenov IV et al. Protective efficacy of IgA monoclonal antibodies to O and H antigens in a mouse model of intranasal challenge with Salmonella enterica serotype enteritidis. Microbes Infect 2004; 6: 901–1010.
14. Phalipon A, Kaufmann M, Michetti P, et al. Monoclonal immunoglobulin A antibody directed against serotype-specific epitope of Shigella flexnerilipopolysaccharide protects against murine experimental shigellosis. J Exp Med 1995; 182: 769–81.
15. Blanchard TG, Czinn SJ, Maurer R et al. Urease-specific monoclonal antibodies prevent Helicobacter felis infection in mice. Infect Immun 1995; 63: 1394–9.
16. Apter FM, Lencer WI, Finkelstein RA et al. Monoclonal immunoglobulin A antibodies directed against cholera toxin prevent the toxin-induced chloride secretory response and block toxin binding to intestinal epithelial cells in vitro. Infect Immun 1993; 61: 5271–8.
17. Stubbe H, Berdoz J, Kraehenbuhl JP, Corthésy B. Polymeric IgA is superior to monomeric IgA and IgG carrying the same variable domain in preventing Clostridium difficile toxin A damaging of T84 monolayers. J Immunol 2000; 164: 1952–60.
18. Mantis NJ, Palaia J, Hessell AJ et al. Inhibition of HIV-1 infectivity and epithelial cell transfer by human monoclonal IgG and IgA antibodies carrying the b12 V region. J Immunol 2007; 179: 3144–52.
19. Perrier C, Sprenger N, Corthésy B. Glycans on secretory component participate in innate protection against mucosal pathogens. J Biol Chem 2006; 281: 14280–7. DOI: 10.1074/jbc.M512958200
20. Boullier S, Tanguy M, Kadaoui KA et al. Secretory IgA-mediated neutralization of Shigella flexneri prevents intestinal tissue destruction by down-regulating inflammatory circuits. J Immunol 2009; 183: 5879–85. DOI: 10.4049/jimmunol.0901838
21. Janzi M, Kull I, Sjöberg R et al. Selective IgA deficiency in early life: association to infections and allergic diseases during childhood. Clin Immunol 2009; 133: 78–85.
22. Zinneman HH, Kaplan AP. The association of giardiasis with reduced intestinal secretory immunoglobulin A. Dig Dis Sci 1975; 125: 207–13.
23. Aghamohammadi A, Cheraghi T, Gharagozlou M et al. IgA deficiency: correlation between clinical and immunological phenotypes. J Clin Immunol 2009; 29: 130–6.
24. Barka N, Shen G-Q, Schonfeld Y et al. Multireactive patterns of serum autoantibodies in asymptomatic individuals with immunoglobulin A deficiency. Clin Diag Lab Immunol 1995; 2: 469–72.
25. Edwards E, Razvi S, Cunningham-Rundles C. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. Clin Immunol 2004; 111: 93–7.
26. Prince HE, Norman GL, Binder WL. Immunoglobulin A (IgA) deficiency and alternative celiac disease-associated antibodies in sera submitted to a reference laboratory for endomysial IgA testing. Clin Diagn Lab Immunol 2000; 7: 192–6.
27. Husby S, Koletzko S, Korponay-Szabo IR et al. European Society for Pediatric Gastroenterology, Hepatology, and Nutrition Guidelines for the Diagnosis of Coeliac Disease. JPGN 2012; 54: 136–60.
28. Jacob CM, Pastorino AC, Fahl K et al. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol 2008; 28: S56–S61.

________________________________________________

1. Geha RS, Notarangelo LD, Casanova JL et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. J Allergy Clin Immunol 2007; 120: 776–94.
2. Yel L. Selective IgA Deficiency. J Clin Immunol 2010; 30 (1): 10–6.
3. Latiff AH, Kerr MA. The clinical significance of immunoglobulin A deficiency. Ann Clin Biochem 2007; 44 (Pt 2): 131–9.
4. Cunningham-Rundles C. Physiology of IgA and IgA deficiency. J Clin Immunol 2001; 21: 303–9.
5. Woof JM, Kerr MA. The function of immunoglobulin A in immunity. J Pathol 2006; 208: 270–82.
6. Russell MW, Sibley DA, Nikolova EB et al. IgA antibody as a non-inflammatory regulator of immunity. Biochem Soc Trans 1997; 25: 466–70.
7. Corthésy B. Roundtrip ticket for secretory IgA: role in mucosal homeostasis? J Immunol 2007; 178: 27–32.
8.Drannik G.N. Clinical immunology and allergology. Moscow: Medical News Agency, 2003 (in Russian).
9. Cerutti A, Rescigno M. The biology of intestinal immunoglobulin A responses. Immunity 2008; 28: 740–50.
10. Bäckhed F, Ley RE, Sonnenburg JL et al. Host-bacterial mutualism in the human intestine. Science 2005; 307: 1915–20.
11. Macpherson AJ, Geuking MB, McCoy KD. Immune responses that adapt the intestinal mucosa to commensal intestinal bacteria. Immunology 2005; 115: 1531–62.
12. Corthésy B. Multi-faceted functions of secretory IgA at mucosal surfaces. Front Immunol 2013; 4: 185.
13. Iankov ID, Petrov DP, Mladenov IV et al. Protective efficacy of IgA monoclonal antibodies to O and H antigens in a mouse model of intranasal challenge with Salmonella enterica serotype enteritidis. Microbes Infect 2004; 6: 901–1010.
14. Phalipon A, Kaufmann M, Michetti P, et al. Monoclonal immunoglobulin A antibody directed against serotype-specific epitope of Shigella flexnerilipopolysaccharide protects against murine experimental shigellosis. J Exp Med 1995; 182: 769–81.
15. Blanchard TG, Czinn SJ, Maurer R et al. Urease-specific monoclonal antibodies prevent Helicobacter felis infection in mice. Infect Immun 1995; 63: 1394–9.
16. Apter FM, Lencer WI, Finkelstein RA et al. Monoclonal immunoglobulin A antibodies directed against cholera toxin prevent the toxin-induced chloride secretory response and block toxin binding to intestinal epithelial cells in vitro. Infect Immun 1993; 61: 5271–8.
17. Stubbe H, Berdoz J, Kraehenbuhl JP, Corthésy B. Polymeric IgA is superior to monomeric IgA and IgG carrying the same variable domain in preventing Clostridium difficile toxin A damaging of T84 monolayers. J Immunol 2000; 164: 1952–60.
18. Mantis NJ, Palaia J, Hessell AJ et al. Inhibition of HIV-1 infectivity and epithelial cell transfer by human monoclonal IgG and IgA antibodies carrying the b12 V region. J Immunol 2007; 179: 3144–52.
19. Perrier C, Sprenger N, Corthésy B. Glycans on secretory component participate in innate protection against mucosal pathogens. J Biol Chem 2006; 281: 14280–7. DOI: 10.1074/jbc.M512958200
20. Boullier S, Tanguy M, Kadaoui KA et al. Secretory IgA-mediated neutralization of Shigella flexneri prevents intestinal tissue destruction by down-regulating inflammatory circuits. J Immunol 2009; 183: 5879–85. DOI: 10.4049/jimmunol.0901838
21. Janzi M, Kull I, Sjöberg R et al. Selective IgA deficiency in early life: association to infections and allergic diseases during childhood. Clin Immunol 2009; 133: 78–85.
22. Zinneman HH, Kaplan AP. The association of giardiasis with reduced intestinal secretory immunoglobulin A. Dig Dis Sci 1975; 125: 207–13.
23. Aghamohammadi A, Cheraghi T, Gharagozlou M et al. IgA deficiency: correlation between clinical and immunological phenotypes. J Clin Immunol 2009; 29: 130–6.
24. Barka N, Shen G-Q, Schonfeld Y et al. Multireactive patterns of serum autoantibodies in asymptomatic individuals with immunoglobulin A deficiency. Clin Diag Lab Immunol 1995; 2: 469–72.
25. Edwards E, Razvi S, Cunningham-Rundles C. IgA deficiency: clinical correlates and responses to pneumococcal vaccine. Clin Immunol 2004; 111: 93–7.
26. Prince HE, Norman GL, Binder WL. Immunoglobulin A (IgA) deficiency and alternative celiac disease-associated antibodies in sera submitted to a reference laboratory for endomysial IgA testing. Clin Diagn Lab Immunol 2000; 7: 192–6.
27. Husby S, Koletzko S, Korponay-Szabo IR et al. European Society for Pediatric Gastroenterology, Hepatology, and Nutrition Guidelines for the Diagnosis of Coeliac Disease. JPGN 2012; 54: 136–60.
28. Jacob CM, Pastorino AC, Fahl K et al. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol 2008; 28: S56–S61.

Авторы

Ю.А. Дмитриева*1,2, И.Н.Захарова1, Е.Р. Радченко3

1 ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования»
Минздрава России, Москва, Россия;
2 ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, Москва, Россия;
3 ГБУЗ «Детская городская клиническая больница им. З.А. Башляевой» Департамента здравоохранения г. Москвы, Москва, Россия
*jadmitrieva@mail.ru

________________________________________________

Iuliia A. Dmitrieva*1,2, Irina N. Zakharova1, Elena R. Radchenko3

1 Russian Medical Academy of Continuous Professional Education, Moscow, Russia;
2 Dmitriy Rogachev National Medical Research Center for Pediatric Hematology, Oncology and Immunology,
Moscow, Russia;
3 Baslyaeva Children's City Clinical Hospital, Moscow, Russia
*jadmitrieva@mail.ru

Назад к списку

Цель портала OmniDoctor – предоставление профессиональной информации врачам, провизорам и фармацевтам.