Primary immune deficiency disease in adults. Preferential disturbance of antibody synthesis
DOI: https://dx.doi.org/10.18565/therapy.2018.7-8.119-124
E.A. Latysheva
«Institute of Immunology» FSBI State Scientific Center of FMBA of Russia
The problem of primary immunodeficiency (PID) in adults becomes more and more important in recent years, as the number of such kind of patients over 18 years of age has been steadily increasing; the number of them amounts to 30–40% of PID registers in different countries. Due to low awareness of this group of diseases, they are characterized by low detectability and late diagnosis. The article discusses the most common forms of PID which are character for persons over 18 years old, also the actual aspects of clinic, diagnosis and treatment are represented.
Keywords: primary immunodeficiency in adults, general variable immune deficiency, selective IgA deficiency, deficiency of IgG subclasses, X-lined immunoglobulinemia, immunoglobulins
Literature
Bruton O.C. Agammaglobulinemia. Pediatrics. 1952; 9(6): 722–27. Verma N., Thaventhiran A., Gathmann B. ESID Registry Working Party, Thaventhiran J., Grimbacher B. Therapeutic Management of Primary Immunodeficiency in Older Patients. Drugs Aging. 2013; 30(7): 503–12. Gathmann B. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J. Allergy Clin. Immunol. 2014; 134(1); 116–26. Driessen G.M., van der Burg М. Educational paper: primary antibody deficiencies. Eur. J. Pediatr. 2011; 170(6): 693–702. Ковальчук Л.В., Ганковская Л.В., Мешкова Р.Я. Клиническая иммунология и аллергология с основами общей иммунологии. М., 2005. Хаитов Р.М. Физиология иммунной системы. М., 2001. 224 с. Picard C., Bobby Gaspar H., Al-Herz W., Bousfiha A., Casanova J.L., Chatila T., Crow Y.J., Cunningham-Rundles C., Etzioni A., Franco J.L., Holland S.M., Klein C., Morio T., Ochs H.D., Oksenhendler E., Puck J., Tang M.L.K., Tangye S.G., Torgerson T.R., Sullivan K.E. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. J. Clin. Immunol. 2018; 38(1): 96–128. Yazdani R., Azizi G.H. Abolhassani, Aghamohammadi A. Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management. Sc. J. Immunol. 2017; 85: 3–12. Moise A., Nedelcu F.D., Toader M.A., Sora S.M., Tica A., Ferastraoaru D.E., Constantinescu I. Primary immunodeficiencies of the B lymophocyte. J. Med. Life. 2010; 3(1): 60–3. Yel L. Selective IgA deficiency. J. Clin. Immunol. 2010; 30(1): 10–6. Picard C., Al-Herz W., Bousfiha A., Casanova J.L., Chatila T., Conley M.E., Cunningham-Rundles C., Etzioni A., Holland S.M., Klein C., Nonoyama S., Ochs H.D., Oksenhendler E.. Puck J.M., Sullivan K.E., Tang M.L., Franco J.L., Gaspar H.B. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015. J. Clin. Immunol. 2015; 35(8): 696–726. Singh K., Chang C., Gershwin M.E. IgA deficiency and autoimmunity. Autoimmun. Rev. 2014; 13(2): 163–77. Jorgensen G.H., Ornolfsson A.E., Johannesson A., Gudmundsson S., Janzi M., Wang N., Hammarström L., Ludviksson B.R. Association of immunoglobulin A deficiency and elevated thyrotropin-receptor autoantibodies in two Nordic countries. Hum. Immunol. 2011; 72: 166–72. Rawat A., Suri D., Gupta A., Saikia B., Minz R.W., Singh S. Isolated Immunoglobulin G4 Subclass Deficiency in a Child with Bronchiectasis Indian J. Pediatr. 2014; 81(9): 932–33. Staines Boone A.T., Torres Martínez M.G., López Herrera G., de Leija Portilla J.O., Espinosa Padilla S.E., Espinosa Rosales F.J., Lugo Reyes S.O. Gastric adenocarcinoma in the context of X-linked agammaglobulinemia: case report and review of the literature. J. Clin. Immunol. 2014; 34(2): 134–37. Jolles S. The variable in common variable immunodeficiency: a disease of complex phenotypes. J. Allergy Clin. Immunol. Pract. 2013; 1(6): 545–56. Ameratunga R., Brewerton M., Slade C., Jordan A., Gillis D., Steele R., Koopmans W., Woon S.T. Comparison of diagnostic criteria for common variable immunodeficiency disorder. Front. Immunol. 2014; 15(5): 415. Perez E.E., Orange J.S., Bonilla F., Chinen J., Chinn I.K., Dorsey M., El-Gamal Y., Harville T.O., Hossny E., Mazer B., Nelson R., Secord E., Jordan S.C., Stiehm E.R., Vo A.A., Ballow M. Update on the use of immunoglobulin in human disease: A review of evidence. J. Allergy Clin. Immunol. 2017; 139(3S): 1–46.
About the Autors
Elena A. Latysheva, PhD, associate professor of the Department of clinical immunology of «N.I. Pirogov RNRMU» FSBEI of HE, Senior research scientist of the Department of immunopathology of the Institute Of Immunology clinic Federal state budgetary institution MRI of FMBA of Russia. Address: 115478, Moscow, 24 Kashirskoye shosse St. Tel: +74996127773. E-mail: ea.latysheva@nrcii.ru