360 INCREASED FREQUENCY OF HLA-B44, -B8, -DR17(3) -DQ2, AND -DR7 -DQ2 IN PATIENTS WITH NORMAL SERUM IMMUNOGLOBULINS WHO SUFFER WITH RECURRENT SINOPULMONARY INFECTIONS. (1st January 2005)

• Record Type: Journal Article
• Authors: Mehaffey, G.; Johnston, D.; Schroeder, H. W.
• Is Part Of: Journal of investigative medicine. Volume 53:Number 1(2005)
• Page Start: S317
• Page End: S317
• Publication Date: 2005-01-01
• Subjects: Clinical medicine -- Periodicals; Medicine -- Research -- Periodicals; Medicine; Research -- United States; Clinical medicine; Medicine -- Research; Periodicals; 616.075
• DOI: 10.2310/6650.2005.00006.359
• Languages: English
• ISSNs: 1081-5589

Abstract

Abstract : We sought to test the hypothesis that the clinical spectrum of the syndrome of selective IgA deficiency (IgAD)/common variable immune deficiency (CVID) might extend to include patients with serum immunoglobulin levels who suffer with recurrent sinopulmonary infections. In our patient population in the southeastern United States, approximately 80% of IgAD/CVID patients have inherited all or part of two extended MHC haplotypes: HLA -A1, -B8, -DR17(3), -DQ2 and HLA -B44, -DR7, -DQ2. We haplotyped 65 consecutive patients who presented to the UAB Adult Primary Immunodeficiency Clinic with a history of unexplained recurrent sinopulmonary infections (RSPI) and compared their clinical history and MHC genotypes to those exhibited by the patients with CVID in our clinic. For RSPI patients, the average IgM level was 125 mg/dL (nl 50-450), IgG 908 mg/dL (nl 700-1500), and IgA 193 mg/dL (nl 60-380). The average age at the time of onset of sinusitis (58 patients) in the RSPI patients was 21 versus 18.5 in 60 patients with CVID. The average age at the onset of bronchitis (39 patients) was 18.5 versus 20.3 in 60 patients with CVID. Average age at the time of their first pneumonia (35 patients) was 21.8 versus 28.5 in 50 with CVID. And the average age at the onset of bronchiectasis (5 patients) was 45 versus 32.2 in 16 with CVID. Together, 44 (68%) of the 65 patients had inherited all or a portion of the two IgAD/CVID susceptibility haplotypes as compared to 50 (80%) of the 63 CVID patients who have been haplotyped (p = .1631, Fisher exact test). By antigen frequency, the most common MHC allele was -B44 (43%), followed by -B8 (26%), -DR17(3) -DQ2 (25%), and -DR7 -DQ2 (17%). When compared to compilations of MHC prevalence in the Caucasian population of North America, these prevalences differed significantly for HLA -B44 (p < .007). These data suggest that the spectrum of IgAD/CVID associated with the two MHC haplotypes noted above may extend to include patients whose immunoglobulins are ostensively normal in concentration, but lacking in efficacy.