PMID- 33048985
OWN - NLM
STAT- MEDLINE
DCOM- 20201204
LR  - 20201214
IS  - 1932-6203 (Electronic)
IS  - 1932-6203 (Linking)
VI  - 15
IP  - 10
DP  - 2020
TI  - Characterization of adult patients with IgG subclass deficiency and subnormal 
      IgG2.
PG  - e0240522
LID - 10.1371/journal.pone.0240522 [doi]
LID - e0240522
AB  - BACKGROUND: Adults with IgG subclass deficiency (IgGSD) with subnormal IgG2 are 
      inadequately characterized. METHODS: We retrospectively analyzed observations in 
      unrelated adults with IgGSD evaluated in a single hematology clinic (1991-2019) 
      and selected those with subnormal serum IgG2 (<117 mg/dL (<1.2 g/L)) without 
      corticosteroid therapy to describe: age; prevalence of women; upper/lower 
      respiratory infection; autoimmune condition(s); atopy; other allergy; frequent or 
      severe respiratory tract infection in first-degree relatives; IgG, IgG 
      subclasses, IgA, and IgM; blood lymphocyte subpopulations; human leukocyte 
      antigen (HLA)-A and -B types and haplotypes; and 23-valent pneumococcal 
      polysaccharide vaccination (PPSV23) responses. We determined the prevalence of 
      subnormal IgG2 among unrelated adults with IgGSD without corticosteroid therapy 
      and compared general characteristics of those with and without subnormal IgG2. 
      RESULTS: There were 18 patients (94.4% women) with subnormal IgG2. Mean age was 
      52 +/- 11 y. Upper/lower respiratory infection occurred in 94.4%/74.8%, 
      respectively. Autoimmune condition(s), atopy, other allergy, and frequent or 
      severe respiratory infection in first-degree relatives occurred in 44.4%, 44.4%, 
      61.1%, and 22.2%, respectively. Median IgG2 was 105 mg/dL (83, 116). Subnormal 
      IgG, IgG1, IgG3, IgG4, IgA, and IgM was observed in 66.7%, 50.0%, 100.0%, 5.6%, 
      33.3%, and 0%, respectively. Lymphocyte subpopulations were normal in most 
      patients. HLA frequencies were similar in patients and controls. Three of 4 
      patients had no protective S. pneumoniae serotype-specific IgG levels before or 
      after PPSV23. These 18 patients represent 7.6% of 236 adults with IgGSD. 
      Prevalence of subnormal IgG, subnormal IgG3, and subnormal IgA was significantly 
      greater in 18 adults with subnormal IgG2 than 218 adults without subnormal IgG2. 
      Prevalence of subnormal IgM was significantly lower in patients with subnormal 
      IgG2. CONCLUSIONS: Characteristics of adults with IgGSD with subnormal IgG2 
      include female predominance, other immunologic abnormalities, subnormal IgG3 
      and/or IgG1, lack of HLA-A and -B association, and suboptimal PPSV23 response.
FAU - Barton, James C
AU  - Barton JC
AUID- ORCID: 0000-0003-2876-8276
AD  - Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, 
      United States of America.
AD  - Southern Iron Disorders Center, Birmingham, Alabama, United States of America.
AD  - Department of Medicine, Brookwood Medical Center, Birmingham, Alabama, United 
      States of America.
FAU - Barton, Jackson C
AU  - Barton JC
AD  - Southern Iron Disorders Center, Birmingham, Alabama, United States of America.
FAU - Bertoli, Luigi F
AU  - Bertoli LF
AD  - Department of Medicine, Brookwood Medical Center, Birmingham, Alabama, United 
      States of America.
FAU - Acton, Ronald T
AU  - Acton RT
AD  - Southern Iron Disorders Center, Birmingham, Alabama, United States of America.
AD  - Department of Microbiology, University of Alabama at Birmingham, Birmingham, 
      Alabama, United States of America.
LA  - eng
PT  - Journal Article
DEP - 20201013
PL  - United States
TA  - PLoS One
JT  - PloS one
JID - 101285081
RN  - 0 (Biomarkers)
RN  - 0 (HLA Antigens)
RN  - 0 (Immunoglobulin G)
SB  - IM
MH  - Adult
MH  - Biomarkers/*blood
MH  - Female
MH  - Follow-Up Studies
MH  - HLA Antigens/metabolism
MH  - Humans
MH  - IgG Deficiency/blood/*epidemiology/pathology
MH  - Immunoglobulin G/*blood
MH  - Incidence
MH  - Lymphocytes/metabolism/pathology
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - ROC Curve
MH  - Respiratory Tract Infections/blood/*epidemiology/pathology
MH  - Retrospective Studies
MH  - Risk Factors
PMC - PMC7553271
COIS- The authors have declared that no competing interests exist.
EDAT- 2020/10/14 06:00
MHDA- 2020/12/15 06:00
PMCR- 2020/10/13
CRDT- 2020/10/13 17:12
PHST- 2020/01/26 00:00 [received]
PHST- 2020/09/29 00:00 [accepted]
PHST- 2020/10/13 17:12 [entrez]
PHST- 2020/10/14 06:00 [pubmed]
PHST- 2020/12/15 06:00 [medline]
PHST- 2020/10/13 00:00 [pmc-release]
AID - PONE-D-20-02354 [pii]
AID - 10.1371/journal.pone.0240522 [doi]
PST - epublish
SO  - PLoS One. 2020 Oct 13;15(10):e0240522. doi: 10.1371/journal.pone.0240522. 
      eCollection 2020.