Newly identified common variable immunodeficiency in an elderly patient with rheumatoid arthritis

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Common variable immunodeficiency is a group of heterogeneous diseases that are primary immunodeficiencies with a predominant lack of antibody synthesis. The most common manifestation is chronic infectious pathology of the respiratory tract, less often — of the gastrointestinal tract, as well as septic arthritis. Non-infectious manifestations include autoimmune diseases, non-infectious diarrhea, cancer, benign lymphoid hyperplasia. In one patient, a combination of several syndromes is possible. Unlike most other primary immunodeficiencies that debut in early childhood, this pathology often manifests in the third or fourth decade of life, and sometimes later in apparently healthy people. Although the routine laboratory method for determining the concentration of the main classes of immunoglobulins makes it quite easy to identify patients with common variable immunodeficiency, often the diagnosis is made many years after the onset of the first clinical symptoms, when serious complications have already developed. The disease is characterized by a gradual onset, chronic progressive course and ineffectiveness of standard therapy. The main method of treatment is lifelong replacement therapy with intravenous immunoglobulins. The article considers a clinical case when an elderly patient with seronegative rheumatoid arthritis with a juvenile onset was diagnosed many years later. The results of immunological studies, including immunophenotyping of peripheral blood and bone marrow lymphocytes, are presented. Issues of differential diagnosis with secondary hypogammaglobulinemia and lymphoproliferative diseases are highlighted. It has been stated that until now, doctors of various specialties are not sufficiently familiar with the problem of primary immunodeficiencies, especially when it comes to adults, and non-infectious manifestations dominate in the clinical picture.

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Oksana V. Moskaletc

Moscow Regional Research Clinical Institute named after M.F. Vladimirsky

Author for correspondence.
ORCID iD: 0000-0002-6118-6465

M.D., Cand. Sci. (Med.), Leading Researcher, Laboratory of Biomedical Research Methods

Russian Federation, Moscow, Russia

Yuliya Ju. Tchuksina

Moscow Regional Research Clinical Institute named after M.F. Vladimirsky

ORCID iD: 0000-0002-4393-1759

M.D., Cand. Sci. (Med.), Senior Researcher, Laboratory of Biomedical Research Methods

Russian Federation, Moscow, Russia


  1. Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, Scheible R, Rusch S, Gasteiger LM, Grimbacher B, Mahlaoui N, Ehl S. The European Society for Immunodeficiencies (ESID) registry working definitions for the clinical diagnosis of inborn errors of immunity. J Allergy Clin Immunol Pract. 2019;6:1763–1770. doi: 10.1016/j.jaip.2019.02.004.
  2. Vasilyeva MM, Sai IA. Primary immunodeficiencies with predominant impairment of antibody synthesis. Public health of the Far East. 2021;(4):24–32. (In Russ.) doi: 10.33454/1728-1261-2021-4-24-32.
  3. Yazdani R, Habibi S, Sharifi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A. Common variable immunodeficiency: Epidemiology, pathogenesis, clinical manifestations, diagnosis, classification and management. J Investig Allergol Clin Immunol. 2020;30(1):14–34. doi: 10.18176/jiaci.0388.
  4. Odnoletkova I, Kindle G, Quinti I, Grimbacher B, Knerr V, Gathmann B, Ehl S, Mahlaoui N, Van Wilder P, Bogaerts K, de Vries E, Plasma Protein Therapeutics Association (PPTA) Taskforce. The burden of common variable immunodeficiency disorders: A retrospective analysis of the European Society for Immunodeficiency (ESID) registry data. Orphanet J Rare Dis. 2018;13(1):201. doi: 10.1186/s13023-018-0941-0.
  5. Donq J, Liaq H, Wen D, Wanq J. Adult common immunodeficiency. Am J Med Sci. 2016;351(3):239–243. doi: 10.1016/j.amjms.2015.12.010.
  6. Sanchez LA, Maggadottir SM, Pantell MS, Lugar P, Cunningham-Rundles C, Sullivan KE. Two sides of the same coin: Pediatric-onset and adult-onset common variable immune deficiency. J Clin Immunol. 2017;37(6):592–602. doi: 10.1007/s10875-017-0415-5.
  7. Ramirez NJ, Posadas-Cantera S, Caballero-Oteyza A, Camacho-Ordonez N, Grimbacher B. There is no gene for CVID — novel monogenetic causes for primary antibody deficiency. Curr Opin Immunol. 2021;72:176–185. doi: 10.1016/j.coi.2021.05.010.
  8. Maglione PJ. Autoimmune and lymphoproliferative complications of common variable immune deficiency. Curr Allergy Asthma Rep. 2016;3:19. doi: 10.1007/s11882-016-0597-6.
  9. Feuille EJ, Anooshiravini N, Sullivan KE, Fuleihan RL, Cunningham-Rundles C. Autoimmune cytopenias and associated conditions in CVID: A report from the USIDNET registry. J Clin Immunol. 2018;38:28–34. doi: 10.1007/s10875-017-0456-9.
  10. Gutierrez MG, Sillivan KE, Fuleihan R; USDNET Concortium; Bingam 3rd CO. Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency. Semin Arthritis Rheum. 2018;48(2):318–326. doi: 10.1016/j.semarthrit.2018.02.013.
  11. Danieli MG, Mezzanotte C, Verga JU, Menghini D, Pedini V, Bilò MB, Moroncini G. Common variable immunodeficiency in elderly patients: A long-term clinical experience. Biomedicines. 2022;10(3):635. doi: 10.3390/biomedicines10030635.
  12. Pikkarainen S, Martelius T, Ristimäki A, Siitonen S, Seppänen MRJ, Färkkilä M. High prevalence of gastrointestinal manifestations in common variable immune deficiency. Am J Gastroenterol. 2019;114:648–655. doi: 10.14309/ajg.0000000000000140.
  13. Irie E, Shirota Y, Suzuki C, Tajima Y, Ishizawa K, Kameoka J, Harigae H, Ishii T. Severe hypogammaglobulinemia persisting for 6 years after treatment with rituximab combined chemotherapy due to arrest of B lymphocyte differentiation together with alteration of T lymphocyte homeostasis. Int J Hematol. 2010;91(3):501–508. DOI: 10/1007/s12185-010-0528-6.
  14. Kado R, Sanders G, McCune WJ. Diagnostic and therapeutic considerations in patients with hypogammaglobulinemia after rituximab therapy. Curr Opin Rheumatol. 2017;29(3);228–233. doi: 10.1097/BOR. 0000000000000377.
  15. Tieu J, Smith RM, Gopaluni S, Kumararatne DS, McClure M, Manson A, Houghton S, Jayne DRW. Rituximab associated hypogammaglobulinemia in autoimmune diseases. Front Immunol. 2021;12:671503. doi: 10.3389/fimmu.2021.671503.
  16. Moskalets OV. Late-onset hypogammaglobulinemia after rituximab the-rapy. Kazan Medical Journal. 2019;100(2):288–294. (In Russ.) doi: 10.17816/KMJ2019-288.
  17. Otani Otani IM, Lehman Lehman HK, Jongco Jongco AM, Tsao Tsao LR, Azar Azar AE, Tarrant Tarrant TK, Engel Engel E, Walter Walter JE, Truong Truong TQ, Khan Khan DA, Ballow Ballow M, Cunningham-Rundles Rundles C, Lu Lu H, Kwan Kwan M, Barmettler Barmettler S. Practical guidance for the diagnosis and management of secondary hypogammaglobulinemia: A Work Group report of the AAAI Primary Immunodeficiency and Altered Immune Response Committees. J Allergy Clin Immunol. 2022;149(5):1525–1560. doi: 10.1016/j.jaci.2022.01.025.
  18. Azizi G, Abolhassani H, Asgardoon MH, Azizi G, Alinia T, Yazdani R, Mohammadi J, Rezaei N, Ochs HD, Aghamohammadi A. Autoimmunity in common variable immunodeficiency: Epidemiology, pathophysiology and management. Expert Rev Clin Immunol. 2017;13(2):101–115. doi: 10.1080/1744666X.2016.1224664.

Supplementary files

Supplementary Files
1. Рис. 1. Рентгенограмма органов грудной клетки пациентки Н.

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