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Immunoglobulin G subclass deficiency is the major phenotype of primary immunodeficiency in a Korean adult cohort.

Kim JH, Park HJ, Choi GS, Kim JE, Ye YM, Nahm DH, Park HS - J. Korean Med. Sci. (2010)

Bottom Line: IgG3 and IgG4 were the most affected subclasses.Bronchial asthma, rhinitis, and several autoimmune diseases were common associated diseases.IgG and IgG subclass deficiency should be considered in adult patients presenting with recurrent upper and lower respiratory infections, particularly in those with respiratory allergies or autoimmune diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Allergy and Rheumatology, Ajou University School of Medicine, Suwon, Korea.

ABSTRACT
Primary immunodeficiency disease (PID) is a rare disorder in adults. Most often, serious forms are detected during infancy or childhood. However, mild forms of PID may not be diagnosed until later in life, and some types of humoral immunodeficiency may occur in adulthood. The purpose of this study was to identify clinical features of PID in Korean adults. A retrospective study was performed on 55 adult patients who were diagnosed as PID between January 1998 and January 2009 at a single tertiary medical center in Korea. IgG subclass deficiency was the most common phenotype (67%, 37/55), followed by total IgG deficiency (20%, 11/55), IgM deficiency (7%, 4/55), common variable immunodeficiency (2%, 1/55), and X-linked agammaglobulinemia (2%, 1/55). IgG3 and IgG4 were the most affected subclasses. Upper and lower respiratory tract infections (76%) were the most frequently observed symptoms, followed by multiple site infection (11%), urinary tract infection, and colitis. Bronchial asthma, rhinitis, and several autoimmune diseases were common associated diseases. IgG and IgG subclass deficiency should be considered in adult patients presenting with recurrent upper and lower respiratory infections, particularly in those with respiratory allergies or autoimmune diseases.

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Distribution of primary immunodeficiency in adult patients from a single medical center in Korea.
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Figure 1: Distribution of primary immunodeficiency in adult patients from a single medical center in Korea.

Mentions: In the present study, 55 adult patients were diagnosed as having PID. All patients were predominantly antibody immunodeficient. In contrast to results in children, none of the patients were identified as having any of the following: combined T- and B-cell immunodeficiency, other well-defined immunodeficiencies, immune dysregulation, and congenital defects in phagocyte number and/or function. Within the predominantly antibody immunodeficiency category, the most common phenotype was IgG subclass deficiency (n=37, 67.3%), followed by total IgG deficiency (n=11, 20.0%), IgM deficiency (n=4, 7.3%), selective IgA deficiency (n=1, 1.8%), common variable immunodeficiency (CVID) (n=1, 1.8%) and X-linked agammaglobulinemia (n=1, 1.8%, Fig. 1). Among patients with IgG subclass deficiency, IgG3 subclass deficiency was the most common (n=17, 47.2%), followed by IgG4 subclass deficiency (n=9, 25.0%). In total, 16.7% of patients with IgG subclass deficiency showed multiple IgG subclass deficiency. Synchronous IgG3 and IgG4 deficiency was most frequently noted. Only two patients showed different disease patterns (IgG1 with IgG4 deficiency and IgG1 with IgG2 deficiency). The mean immunoglobulin values in patients with IgG subclass deficiency were as follows: IgG, 1018.6±380.4 mg/dL; IgA, 226.2±117.7 mg/dL; IgM, 147.5±110.6 mg/dL; IgG1, 628.6±360.7 mg/dL; IgG2, 392.4±189.9 mg/dL; IgG3, 29.5±25.0 mg/dL; and IgG4, 28.9±28.8 mg/dL.


Immunoglobulin G subclass deficiency is the major phenotype of primary immunodeficiency in a Korean adult cohort.

Kim JH, Park HJ, Choi GS, Kim JE, Ye YM, Nahm DH, Park HS - J. Korean Med. Sci. (2010)

Distribution of primary immunodeficiency in adult patients from a single medical center in Korea.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2877226&req=5

Figure 1: Distribution of primary immunodeficiency in adult patients from a single medical center in Korea.
Mentions: In the present study, 55 adult patients were diagnosed as having PID. All patients were predominantly antibody immunodeficient. In contrast to results in children, none of the patients were identified as having any of the following: combined T- and B-cell immunodeficiency, other well-defined immunodeficiencies, immune dysregulation, and congenital defects in phagocyte number and/or function. Within the predominantly antibody immunodeficiency category, the most common phenotype was IgG subclass deficiency (n=37, 67.3%), followed by total IgG deficiency (n=11, 20.0%), IgM deficiency (n=4, 7.3%), selective IgA deficiency (n=1, 1.8%), common variable immunodeficiency (CVID) (n=1, 1.8%) and X-linked agammaglobulinemia (n=1, 1.8%, Fig. 1). Among patients with IgG subclass deficiency, IgG3 subclass deficiency was the most common (n=17, 47.2%), followed by IgG4 subclass deficiency (n=9, 25.0%). In total, 16.7% of patients with IgG subclass deficiency showed multiple IgG subclass deficiency. Synchronous IgG3 and IgG4 deficiency was most frequently noted. Only two patients showed different disease patterns (IgG1 with IgG4 deficiency and IgG1 with IgG2 deficiency). The mean immunoglobulin values in patients with IgG subclass deficiency were as follows: IgG, 1018.6±380.4 mg/dL; IgA, 226.2±117.7 mg/dL; IgM, 147.5±110.6 mg/dL; IgG1, 628.6±360.7 mg/dL; IgG2, 392.4±189.9 mg/dL; IgG3, 29.5±25.0 mg/dL; and IgG4, 28.9±28.8 mg/dL.

Bottom Line: IgG3 and IgG4 were the most affected subclasses.Bronchial asthma, rhinitis, and several autoimmune diseases were common associated diseases.IgG and IgG subclass deficiency should be considered in adult patients presenting with recurrent upper and lower respiratory infections, particularly in those with respiratory allergies or autoimmune diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Allergy and Rheumatology, Ajou University School of Medicine, Suwon, Korea.

ABSTRACT
Primary immunodeficiency disease (PID) is a rare disorder in adults. Most often, serious forms are detected during infancy or childhood. However, mild forms of PID may not be diagnosed until later in life, and some types of humoral immunodeficiency may occur in adulthood. The purpose of this study was to identify clinical features of PID in Korean adults. A retrospective study was performed on 55 adult patients who were diagnosed as PID between January 1998 and January 2009 at a single tertiary medical center in Korea. IgG subclass deficiency was the most common phenotype (67%, 37/55), followed by total IgG deficiency (20%, 11/55), IgM deficiency (7%, 4/55), common variable immunodeficiency (2%, 1/55), and X-linked agammaglobulinemia (2%, 1/55). IgG3 and IgG4 were the most affected subclasses. Upper and lower respiratory tract infections (76%) were the most frequently observed symptoms, followed by multiple site infection (11%), urinary tract infection, and colitis. Bronchial asthma, rhinitis, and several autoimmune diseases were common associated diseases. IgG and IgG subclass deficiency should be considered in adult patients presenting with recurrent upper and lower respiratory infections, particularly in those with respiratory allergies or autoimmune diseases.

Show MeSH
Related in: MedlinePlus