Common variable immunodeficiency disorder: a clinical case

Primary immunodeficiency is a rare congenital pathology associated with failure of immune system, manifested by disturbances of its functions. These defects lead to increased susceptibility of patients to various infectious agents, as well as the development of autoimmune, malignant and other diseas...

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Autores principales:	E. A. Sobko, I. V. Demko, I. A. Soloveva, A. Yu. Kraposhina, N. V. Gordeeva, D. A. Anikin, N. S. Pronkina, O. P. Ischenko
Formato:	article
Lenguaje:	RU
Publicado:	SPb RAACI 2021
Materias:	primary immunodeficiency common variable immunodeficiency disorder hypogammaglobulinemia immunogram normal human immunoglobulin Immunologic diseases. Allergy RC581-607
Acceso en línea:	https://doaj.org/article/2b1378ce321249a5a0c6737491895de9
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spelling	oai:doaj.org-article:2b1378ce321249a5a0c6737491895de92021-11-18T08:03:50ZCommon variable immunodeficiency disorder: a clinical case1563-06252313-741X10.15789/1563-0625-CVI-2089https://doaj.org/article/2b1378ce321249a5a0c6737491895de92021-03-01T00:00:00Zhttps://www.mimmun.ru/mimmun/article/view/2089https://doaj.org/toc/1563-0625https://doaj.org/toc/2313-741XPrimary immunodeficiency is a rare congenital pathology associated with failure of immune system, manifested by disturbances of its functions. These defects lead to increased susceptibility of patients to various infectious agents, as well as the development of autoimmune, malignant and other diseases. Primary immunodeficiency is classified as a rare disease, which was previously associated with a poor prognosis with a high risk of mortality in childhood. To date, the emergence of highly effective treatment methods has changed the course and prognosis of these diseases. Clinicians of various specialties increasingly meet with this pathology in everyday practice, including adult age cohorts. In this regard, early diagnosis of primary immunodeficiency in adults becomes relevant, being associated with choosing optimal therapy, prevention of severe internal organ damage, determination of management strategy for the patient, as well as the need to identify inherited disorders and provide information to the patient’s family. Delayed verification of the diagnosis may cause disability of the patient and development of irreversible, often fatal complications. This article presents our own clinical case with a newly diagnosed clinical condition: Common variable immunodeficiency disorder (CVID), the most common form of primary immunodeficiency in adults. The symptoms of common variable immunodeficiency disorder appear in these patients in adulthood, but a high-quality collected history of the disease will allow you to trace symptoms in the patients even since early childhood. There is a common gap for several years between the onset of the disease and clinical diagnosis, since erroneous diagnosis is often made due to non-specific clinical symptoms that resemble other, more frequent diseases. The prognosis of patients with CVID depends on several factors: frequency of infections, structural disorders in the lungs, the occurrence of autoimmune diseases and the success of infection prevention. Thus, a variety of clinical forms of primary immunodeficiency, lack of awareness of doctors about this pathology, complexity of immunological examination in the general medical network lead to the fact that CVID is not diagnosed for long terms, and patients do not receive the necessary pathogenetic therapy. There is a need for drawing attention of doctors of various disciplines to the fact that the recurrent inflammatory processes of various localization, which are difficult to respond to adequate traditional therapy, may be caused by changes in the immune system, including congenital, genetically determined immunodeficiency.E. A. SobkoI. V. DemkoI. A. SolovevaA. Yu. KraposhinaN. V. GordeevaD. A. AnikinN. S. PronkinaO. P. IschenkoSPb RAACIarticleprimary immunodeficiencycommon variable immunodeficiency disorderhypogammaglobulinemiaimmunogramnormal human immunoglobulinImmunologic diseases. AllergyRC581-607RUMedicinskaâ Immunologiâ, Vol 23, Iss 1, Pp 185-190 (2021)
institution	DOAJ
collection	DOAJ
language	RU
topic	primary immunodeficiency common variable immunodeficiency disorder hypogammaglobulinemia immunogram normal human immunoglobulin Immunologic diseases. Allergy RC581-607
spellingShingle	primary immunodeficiency common variable immunodeficiency disorder hypogammaglobulinemia immunogram normal human immunoglobulin Immunologic diseases. Allergy RC581-607 E. A. Sobko I. V. Demko I. A. Soloveva A. Yu. Kraposhina N. V. Gordeeva D. A. Anikin N. S. Pronkina O. P. Ischenko Common variable immunodeficiency disorder: a clinical case
description	Primary immunodeficiency is a rare congenital pathology associated with failure of immune system, manifested by disturbances of its functions. These defects lead to increased susceptibility of patients to various infectious agents, as well as the development of autoimmune, malignant and other diseases. Primary immunodeficiency is classified as a rare disease, which was previously associated with a poor prognosis with a high risk of mortality in childhood. To date, the emergence of highly effective treatment methods has changed the course and prognosis of these diseases. Clinicians of various specialties increasingly meet with this pathology in everyday practice, including adult age cohorts. In this regard, early diagnosis of primary immunodeficiency in adults becomes relevant, being associated with choosing optimal therapy, prevention of severe internal organ damage, determination of management strategy for the patient, as well as the need to identify inherited disorders and provide information to the patient’s family. Delayed verification of the diagnosis may cause disability of the patient and development of irreversible, often fatal complications. This article presents our own clinical case with a newly diagnosed clinical condition: Common variable immunodeficiency disorder (CVID), the most common form of primary immunodeficiency in adults. The symptoms of common variable immunodeficiency disorder appear in these patients in adulthood, but a high-quality collected history of the disease will allow you to trace symptoms in the patients even since early childhood. There is a common gap for several years between the onset of the disease and clinical diagnosis, since erroneous diagnosis is often made due to non-specific clinical symptoms that resemble other, more frequent diseases. The prognosis of patients with CVID depends on several factors: frequency of infections, structural disorders in the lungs, the occurrence of autoimmune diseases and the success of infection prevention. Thus, a variety of clinical forms of primary immunodeficiency, lack of awareness of doctors about this pathology, complexity of immunological examination in the general medical network lead to the fact that CVID is not diagnosed for long terms, and patients do not receive the necessary pathogenetic therapy. There is a need for drawing attention of doctors of various disciplines to the fact that the recurrent inflammatory processes of various localization, which are difficult to respond to adequate traditional therapy, may be caused by changes in the immune system, including congenital, genetically determined immunodeficiency.
format	article
author	E. A. Sobko I. V. Demko I. A. Soloveva A. Yu. Kraposhina N. V. Gordeeva D. A. Anikin N. S. Pronkina O. P. Ischenko
author_facet	E. A. Sobko I. V. Demko I. A. Soloveva A. Yu. Kraposhina N. V. Gordeeva D. A. Anikin N. S. Pronkina O. P. Ischenko
author_sort	E. A. Sobko
title	Common variable immunodeficiency disorder: a clinical case
title_short	Common variable immunodeficiency disorder: a clinical case
title_full	Common variable immunodeficiency disorder: a clinical case
title_fullStr	Common variable immunodeficiency disorder: a clinical case
title_full_unstemmed	Common variable immunodeficiency disorder: a clinical case
title_sort	common variable immunodeficiency disorder: a clinical case
publisher	SPb RAACI
publishDate	2021
url	https://doaj.org/article/2b1378ce321249a5a0c6737491895de9
work_keys_str_mv	AT easobko commonvariableimmunodeficiencydisorderaclinicalcase AT ivdemko commonvariableimmunodeficiencydisorderaclinicalcase AT iasoloveva commonvariableimmunodeficiencydisorderaclinicalcase AT ayukraposhina commonvariableimmunodeficiencydisorderaclinicalcase AT nvgordeeva commonvariableimmunodeficiencydisorderaclinicalcase AT daanikin commonvariableimmunodeficiencydisorderaclinicalcase AT nspronkina commonvariableimmunodeficiencydisorderaclinicalcase AT opischenko commonvariableimmunodeficiencydisorderaclinicalcase
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Common variable immunodeficiency disorder: a clinical case

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