Selective Immunoglobulin A Deficiency Associated with Immunoglobulin G4 Deficiency in Adult Patient
https://doi.org/10.31550/1727-2378-2024-23-1-82-85
Abstract
Aim. To demonstrate the possibility of developing primary immunodeficiency with impaired antibody synthesis in an adult patient and discuss the algorithm for laboratory diagnostics and treatment tacticsto demonstrate the possibility of developing primar hypoimmunoglobulinemia after a course of imunosupressive therapy.
Key points. Primary immunodeficiencies are a group of diseases associated with monogenic mutations. They do not have a typical clinical picture. A clinical observation is presented, when a selective deficiency of immunoglobulin A in combination with a selective deficiency of the subclass of immunoglobulin G was detected in a patient with chronic bronchopulmonary pathology. Clinical and laboratory criteria for diagnosis and treatment tactics are discussed.
Сonclusion. Primary immunodeficiencies are often hidden by infectious masks. It is necessary to study serum immunoglobulins, with their normal levels or selective deficiency of immunoglobulin A, to additionally investigate the content of subclasses of immunoglobulin G.
About the Author
O. V. Moskalets
M.F. Vladimirsky Moscow Regional Research Clinical Institute
Russian Federation
Russian Federation
61/2 Schepkina Str., build. 1, Moscow, 129110
References
1. Bousfiha A., Jeddane L., Picard C., Al-Herz W. et al. Human inborn errors of immunity: 2019 update of the IUIS phenotypical classification. J. Clin. Immunol. 2020;40(1):66–81. DOI: 10.1007/s10875-020-00758-x
2. Seidel M.G., Kindle G., Gathmann B., Quinti I. et al. The European society for immunodeficiencies (ESID) registry working definitions for the clinical diagnosis of inborn errors of immunity. J. Allergy Clin. Immunol. Pract. 2019;7(6):1763–70. DOI: 10.1016/j.jaip.2019.02.004
3. Kuzmenko N.B., Shcherbina A.Yu. Classification of primary immunodeficiencies as a reflection of modern ideas about their pathogenesis and therapeutic approaches. Russian Journal of Pediatric Hematology and Oncology. 2017;4(3):51–7. (in Russian). DOI: 10.17650/2311-1267-2017-4-3-51-57
4. Gupta A. Primary immunodeficiency disorders: where do we stand? Indian J. Pediatr. 2019;86 (10):873–4. DOI: 10.1007/s12098-01903031-1
5. Vasilyeva M.M., Sai I.A. Primary immunodefi ciencies with predominant impairment of antibody synthesis. Public Health of the Far East. 2021;4:24–32. (in Russian). DOI: 10.33454/1728-1261-2021-4-24-32
6. Yagudina L.A., Khakimova D.M. Common variable immune deficiency in clinical practice. Kazan Medical Journal. 2015;96(2):249–52. (in Russian). DOI: 10.17750/KMJ2015-249
7. Swain S., Selmi C., Gershwin M.E., Teuber S.S. The clinical implications of selective IgA deficiency. J. Transl. Autoimmun. 2019;2:10025. DOI: 10.1016/j.jtaut.2019.100025
8. Moskalets O.V. A case of selective immunoglobulin A deficiency associated with autoimmune gastritis. Almanac of Clinical Medicine. 2016;44(6):790–5. (in Russian). DOI: 10.18786/2072-0505-2016-44-6-790-795
9. Chuchalin A.G. IgG-related diseases. Pulmonologiya. 2017;27(3):311–19. (in Russian). DOI: 10.18093/0869-0189-2017-27-3-311-319
10. Parker A.R., Shold M., Ramsden D.B., Ocejo-Vinyals J.G. et.al. The clinical utility of measuring IgG subclass immunoglobulins during immunological investigation for suspected primary antibody deficiencies. Lab. Medicine. 2017;48(4):314–25. DOI: 10.1093/labmed/lmx058
11. Barton J., Barton C., Bertoli L. Duration of frequent or severe respiratory tract infectionin adults before diagnosis of IgG subclass deficiency. PLoS One. 2019;14(5):e0216940. DOI: 10.1371/journal pone.026940
12. Lee S-H., Ban G-Y., Kim S-C., Chung C-G. et al. Association between primary immunodeficiency and asthma exacerbationin adult asthmatics. Korean J. Intern. Med. 2020;35(2):449–46. DOI: 10.3904/kjm.2018.413
13. Kim J-H., Jang J-H., Lee S-Y., Yang E-M., Jang S.H. et al. Specific antibody deficiency in adult patients with IgG or IgG subclass deficiency. Allergy Asthma Immunol. Res. 2021;13(2):271-83. DOI: 10.4168/aaar.2021.13.2.271
14. Shin J.J., Liauw D., Siddiqui S., Lee J. et al. Immunological and clinical phenotyping in primary antibody deficiencies: a growing disease spectrum. J. Clin. Immunol. 2020;40(4):592–601. DOI: 10.1007/s10875-020-00773-y
15. Barton J.C., Barton J.C., Bertoli L.F., Acton R.T. Characterization of adult patients with IgG subclassdeficiency and subnormal IgG2. PLoS One. 2020;15(10):e0240522. DOI: 10.1371/journal.pone.0240552
16. Modell V., Orange J.S., Quinn J., Modell F. Global report of primary immunodeficiencies: 2018 update from the Jeffrey Model Centers Network on disease classification, regional trends, treatment modalities and physician reported outcomes. Immunol. Res. 2018;66(3):367–80. DOI: 10.1017/s12026-018-8996-5
17. Donq J., Liaq H., Wen D., Wanq J. Adult common immunodeficiency. Am. J. Med. Sci. 2016;351(3):239–43. DOI: 10.1016/j.amjms.2015.12.010
18. Abrahamian J., Agrawal S., Gupta S. Immunological and clinical profile of adult patients with selective immunoglobulin subclass deficiency: response to intravenous immunoglobulin therapy. Clin. Exp. Immunol. 2010;159(3):344–50. DOI: 10.1111/j.1365-2249.2009.04062-x
19. Smith B.M., Buddle I.K., deVries E., Berge I.J.M. et al. Immunoglobulin replacementtherapy versus antibiotic prophylaxis as treatment for incomplete primary antibody deficiency. J. Clin. Immunol. 2021;41(2):382–92. DOI: 10.1007/s108575-020-00841-3
Review
For citations:
Moskalets O.V. Selective Immunoglobulin A Deficiency Associated with Immunoglobulin G4 Deficiency in Adult Patient. Title. 2024;23(1):82-85. (In Russ.) https://doi.org/10.31550/1727-2378-2024-23-1-82-85
Views:
9
Email this article 