Sex: U
Vaccine: PPV
Symptoms: Vaccination failure
Clinical narrative: This published literature report refers to patients with Common variable immunodeficiency (CVID) and immunoglobulin (Ig) G subclass deficiency (IgGSD) are heterogeneous disorders characterized by respiratory tract infections, selective Ig isotype deficiencies, and impaired antibody responses to polysaccharide antigens. (Patients concurrent conditions included autoimmune disorder like Sjogren's syndrome, Hashimoto's thyroiditis, Rheumatoid arthritis, Gastrointestinal tract, Multiple sclerosis, Positive ANA, Psoriatic arthritis, Autoimmune hearing loss, Autoimmune hemolytic anemia, Diabetes mellitus, type 1, Grave's disease, Inflammatory arthritis, Interstitial cystitis, Mixed connective tissue disorder, Myasthenia gravis, Polymyalgia rheumatic, Raynaud's phenomenon, Reiter's uveitis, Sarcoidosis, Skin and mucous membranes, Systemic lupus erythematous, Temporal arteritis and Thrombotic disorders). Inclusion criteria included frequent or severe infections uncontrolled by antibiotic therapy and other management and evidence of hypogammaglobulinemia. Patient with low IgG and normal IgA were accepted as having IgGSD. Each of the present patients diagnosed herein to have IgGSD had primary immunodeficiency with non protective serotype specific serum IgG levels for or impaired response to Streptococcus pneumonia polysaccharide antigen. Using univariable analyses, observations in 34 CVID and 398 IgGSD adult index patients (81.9% women) referred to a hematology/oncology practice were evaluated. Similarities included specialties of referring physicians, mean ages, proportions of women, reactivity to PNEUMOVAX, median serum IgG4 and IgG4 levels, median blood CD56+/CD16+ lymphocyte levels, positivity for HLA-A and B types, and frequencies of selected HLA-A, -B haplotypes. Dissimilarities included greater prevalence of autoimmune conditions, lower median IgG, IgA, and IgM, and lower median CD 19+, CD3+/CD4+, and CD3+/CD8+ blood lymphocytes in CVID patients. Prevalence of Sjogren's syndrome and hypothyroidism was significantly greater in CVID patients. Combined subnormal IgG1/IgG3 occurred in 59% and 29% of CVID and IgGSD patients, respectively. Isolated subnormal IgG3 occurred in 121 IgGSD patients (88% women). Logistic regression on CVID (versus IgGSD) revealed a significant positive association with autoimmune conditions and significant negative associations with IgG1, IgG3, and IgA and CD56+/CD16+ lymphocyte levels, but the odds ratio was increased for autoimmune conditions alone (6.9 (95% C1 1.3, 35.5)). This report refers to 432 adult patients. On an unknown date, patients were vaccinated with PNEUMOVAX 23 (strength, dose, route of administration). Pre and post Panels of pre- and post- pneumococcal vaccine, polyvalent S. pneumonia serotype-specific (measured for at least 4 weeks after initiation of vaccination) IgG antibodies were available in 28 CVID patients (82.5%) and in 315 IgGSD patients (79/1%) (P=0.6572). No CVID patient had pre and post-pneumococcal vaccine, polyvalent antibody test panels in which 100% of serotype antibodies were "protective:' In 26 IgGSD patients (8.3%), pre and post- pneumococcal vaccine, polyvalent antibody panels included 100% protective levels of serotype antibodies (1 versus 8.3%, P=0.1000). There was no increment in the number of protective IgG levels after PNEUMOVAX in 7 CVID patients (25.0%) and in 114 IgGSD patients (36.2%) (Vaccination failure). In CVID patients, distributions of IgG, IgG1, and IgG3levels were normal. Other Ig levels were not normally disturbed. In IgGSD patients, levels of all Ig isotypes were normally disturbed. Correlations of IgG with the sum of IgG1-4 levels were determined. In CVID patients, the Pearson correlation coefficient was 0.4766 (P <0.0001). In IgGSD patients, the correlation coefficient was 0.7688 (P <0.0001). In both CVID and IgGSD patients, the median IgG was slightly greater than the corresponding median of the sum of IgG1-4 (5.51 g/L versus 5.26 g/L, CVID; 7.58 g/L versus 7.49 g/L, IgGSD). Median levels of all Ig isotypes were significantly lower in CVID patients except IgG3 and IgG4 which did not differ significantly between CVID and IgGSD patients. Outcome of the events was unknown. The author considered the event vaccination failure to be related to PNEUMOVAX 23. A copy of the published article is attached as further documentation of the subject's experience. Additional information has been requested.