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1-Antitrypsin Deficiency With and Without Bronchiectasis*
* From the Department of Pneumology (Drs. Miravitlles, de Gracia, Vendrell, Vidal, and Morell) and the Department of Biochemistry (Drs. Rodrigo and Cruz), Immunology Unit, Hospital General Vall d'Hebron, Barcelona, Spain. Supported by Fondo de Investigaciones Sanitarias (FIS) grant 93/0482.
Correspondence to: Marc Miravitlles, MD, PhD, Rocafort 173177, 3°1a, 08015 Barcelona, Spain; e-mail: marcm{at}hg.vhebron.es
Objective: To assess the specific antibody response
against polyvalent pneumococcal vaccine in patients with
1-antitrypsin deficiency (AATD) and respiratory
infections.
Design and participants: We investigated specific IgG, IgG1, and IgG2 antibody responses against the 23-valent antipneumococcal vaccine in 18 patients with AATD phenotype PiZZ, 9 of whom had bronchiectasis and 4 a history of recurrent pneumonia, and compared them with a control group of 40 healthy volunteers.
Interventions: Blood samples were drawn just prior to and 3 weeks after immunization.
Measurements and results: Quantification of specific IgG and its subclasses was performed by an enzyme-linked immunosorbent assay. For patients with AATD, mean increases in specific antipneumococcal titers were 4.7-fold (25 to 75% quartiles, 2.5- to 6.8-fold) for total IgG, 3.2-fold (1.2- to 4.9-fold) for IgG1, and 2.1-fold (1.8- to 3.7-fold) for IgG2. For the control group, the values were 3.3-fold (1.8- to 5.8-fold) for total IgG, 2.5-fold (1.9- to 3.4-fold) for IgG1, and 3.1-fold (1.9- to 4.5-fold) for IgG2; differences were not significant. Patients with bronchiectasis showed a tendency toward higher levels of IgG subclasses than both control subjects and patients without bronchiectasis; however, there was a tendency toward lower postvaccination serum levels of specific antipneumococcal IgG, IgG1, and IgG2 in patients with bronchiectasis compared with patients without bronchiectasis, but this trend did not reach statistical significance. Three of the four patients with recurrent pneumonia did not show an appropriate IgG2 response.
Conclusions: These results suggest that, as a group, patients with AATD have a preserved antibody response against pneumococcal polysaccharides. Patients with bronchiectasis show a tendency toward a decreased antibody response, even with increased serum levels of most Ig types. Individuals with an impaired IgG2 response seem to be at increased risk of recurrent pneumonia. Considering the pernicious effect of pulmonary infections on these patients and the preserved antibody response in a majority of them, pneumococcal vaccination should be recommended to patients with AATD.
Key Words: antibody response
1-antitrypsin deficiency bronchiectasis IgG subclasses pneumococcal vaccine
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