가족성 IgG3 결핍 2예
Familial IgG3 subclass deficiency: A report of two cases
2
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Primary immunodeficiency in adults is thought to be underestimated in Korea. IgG subclass deficiency, defined as a deficiency at least among the 4 subtypes of IgG subclass, IgG1, IgG2, IgG3, and IgG4, results in recurrent infections, in which IgG3 subclass deficiency (IgGSD) is the most commonly found in adult asthmatic patients. Herein, we report 2 cases of familial IgG3SD. In family 1, a female patient aged 17 years with allergic rhinitis (AR) had recurrent upper respiratory infections (URIs), and gastroenteritis. Her mother aged 50 with AR had recurrent URI, otitis media, urinary tract infection, gastroenteritis, and oral ulcer. A younger sister aged 9 years with AR and asthma had recurrent URI almost all the year round. The serum IgA level was found to be lower than the normal level in her mother and the patient, and IgG3 was decreased in the patient and her sister. The IgG3 level of mother showed within the lower normal limit. In family 2, the mother aged 39 years with nonallergic rhinitis had recurrent URI and oral ulcer. Her son aged 16 years and daughter aged 13 years with AR and asthma had recurrent URI, resulting in uncontrolled asthma. Family 2 was found to be IgG3 deficiency in mother and son, and IgA deficiency in son. The IgG3 level of daughter was within the lower normal level. Mother and the first daughter in family 1 as well as mother and son in family 2 was treated with intravenous immunoglobulin, and their recurrent URI reduced. We reported 2 cases of familial IgG3SD.
Abstract
Primary immunodeficiency in adults is thought to be underestimated in Korea. IgG subclass deficiency, defined as a deficiency at least among the 4 subtypes of IgG subclass, IgG1, IgG2, IgG3, and IgG4, results in recurrent infections, in which IgG3 subclass deficiency (IgGSD) is the most commonly found in adult asthmatic patients. Herein, we report 2 cases of familial IgG3SD. In family 1, a female patient aged 17 years with allergic rhinitis (AR) had recurrent upper respiratory infections (URIs), and gastroenteritis. Her mother aged 50 with AR had recurrent URI, otitis media, urinary tract infection, gastroenteritis, and oral ulcer. A younger sister aged 9 years with AR and asthma had recurrent URI almost all the year round. The serum IgA level was found to be lower than the normal level in her mother and the patient, and IgG3 was decreased in the patient and her sister. The IgG3 level of mother showed within the lower normal limit. In family 2, the mother aged 39 years with nonallergic rhinitis had recurrent URI and oral ulcer. Her son aged 16 years and daughter aged 13 years with AR and asthma had recurrent URI, resulting in uncontrolled asthma. Family 2 was found to be IgG3 deficiency in mother and son, and IgA deficiency in son. The IgG3 level of daughter was within the lower normal level. Mother and the first daughter in family 1 as well as mother and son in family 2 was treated with intravenous immunoglobulin, and their recurrent URI reduced. We reported 2 cases of familial IgG3SD.
참고문헌 (16)
- Bonilla FA, "Practice parameter for the diagnosis and management of primary immunodeficiency", J Allergy Clin Immunol, 136, pp.1186~1205 (2015)[DOI]
- Pan-Hammarstrom Q, "Antibody deficiency diseases", Eur J Immunol, 38, pp.327~333 (2008)[DOI]
- Buckley RH, "Immunoglobulin G subclass deficiency: fact or fancy?", Curr Allergy Asthma Rep, 2, pp.356~360 (2002)[DOI]
- Abrahamian F, "Immunological and clinical profile of adult patients with selective immunoglobulin subclass deficiency: response to intravenous immunoglobulin therapy", Clin Exp Immunol, 159, pp.344~350 (2010)[DOI]
- Barton JC, "Selective subnormal IgG3 in 121 adult index patients with frequent or severe bacterial respiratory tract infections", Cell Immunol, 299, pp.50~57 (2016)[DOI]
- [URL]
- 김주희, "Immunoglobulin G Subclass Deficiency is the Major Phenotype of Primary Immunodeficiency in a Korean Adult Cohort", Journal of Korean Medical Science, 25(6), pp.824~828 (2010), 대한의학회
- Ozkan H, "IgA and", or IgG subclass deficiency in children with recurrent respiratory infections and its relationship with chronic pulmonary damage, 2005, pp.15, 69~74
- 김주희, "Immunoglobulin G Subclass Deficiencies in Adult Patients with Chronic Airway Diseases", Journal of Korean Medical Science, 31(10), pp.1560~1565 (2016), 대한의학회
- Soderstrom R, "Effect of immunoglobulin prophylaxis in infection-prone adults with low IgG-subclass levels, a single-blind crossover study", 197~201 (1996)
- Olinder-Nielsen AM, "Immunoglobulin prophylaxis in 350 adults with IgG subclass deficiency and recurrent respiratory tract infections: a long-term follow-up", Scand J Infect Dis, 39, pp.44~50 (2007)[DOI]
- 김주희, "Effects of Immunoglobulin Replacement on Asthma Exacerbation in Adult Asthmatics with IgG Subclass Deficiency", Allergy, Asthma & Immunology Research, 9(6), pp.526~533 (2017), 대한천식알레르기학회
- Barlan IB, "Therapy for patients with recurrent infections and low serum IgG3 levels", J Allergy Clin Immunol, 92, pp.353~355 (1993)[DOI]
- Bonagura VR, "Using intravenous immunoglobulin (IVIG) to treat patients with primary immune deficiency disease", J Clin Immunol, 33(Suppl 2), pp.S90~S94 (2013)[DOI]
- Vidarsson G, "IgG subclasses and allotypes: from structure to effector functions", Front Immunol, 5, pp.520~ (2014)
- Ohnishi H, "A case of selective IgG subclass deficiency with STAT3 mutation", Allergol Int, 65, pp.495~497 (2016)[DOI]