Immunoglobulin G subclass deficiency is the major phenotype of primary immunodeficiency in a Korean adult cohort.
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.
Department of Allergy and Rheumatology, Ajou University School of Medicine, Suwon, Korea.
Joo-Hee
JH
Kim
Han-Jung
HJ
Park
Gil-Soon
GS
Choi
Jeong-Eun
JE
Kim
Young-Min
YM
Ye
Dong-Ho
DH
Nahm
Hae-Sim
HS
Park
D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000175:diagnosis / Q000453:epidemiology; D005260:Female; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000175:diagnosis / Q000453:epidemiology; D017099:IgG Deficiency / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D056910:Republic of Korea / Q000453:epidemiology; D012141:Respiratory Tract Infections / Q000209:etiology; D012189:Retrospective Studies; D053632:X-Linked Combined Immunodeficiency Diseases / Q000175:diagnosis / Q000453:epidemiology