Chest
Clinical InvestigationsAIRWAY DISEASESpecific Antibody Response Against the 23-Valent Pneumococcal Vaccine in Patients With α1-Antitrypsin Deficiency With and Without Bronchiectasis
Section snippets
Study Population
The study population consisted of patients with severe AATD, phenotype PiZZ, seen at our center.
Their results were compared with those of a control group comprised of 40 healthy, nonrelated volunteers (20 women, 20 men; age range, 21 to 48 years; mean age, 30 years), whose characteristics have been described previously.11
None of the patients in either group had a history of prior pneumococcal immunization or known immunodeficiency. All subjects gave informed consent, and the study was approved
Patient Characteristics
The characteristics of the study population consisting of 18 AATD individuals with the PiZZ phenotype are shown in Tables 1and 2 . On CT examination, 15 had pulmonary emphysema and nine had bronchiectasis. Fourteen (78%) suffered from recurrent exacerbations with increased dyspnea, sputum volume, and sputum purulence. Six patients (33%) had a history of previous pneumonia, which was recurrent in four (one of them with small diffuse bronchiectasis). Eight patients were receiving augmentation
Discussion
Patients with AATD often develop infectious respiratory complications. Half of the patients described here had bronchiectasis seen on CT examination. One third had a history of documented pneumonia, which was recurrent in four cases. The majority (14/18; 78%) frequently experienced infectious exacerbations of their COPD.
However, the specific IgG, IgG1, and IgG2 antipneumococcal antibody responses in most patients with AATD did not differ significantly from the responses observed in healthy
References (33)
- et al.
The alpha-1-antitrypsin gene and its mutations: clinical consequences and strategies for therapy
Chest
(1989) - et al.
The role of alpha-1-antitrypsin deficiency in the pathogenesis of immune disorders
Clin Immunol Immunopathol
(1985) - et al.
Evaluación del tratamiento sustitutivo del enfisema por déficit de alfa-1-antitripsina [Assessment of alpha-1-antitrypsin augmentation therapy for emphysema]
Arch Bronconeumol
(1994) - et al.
The amplifier role of T cells in the human in vitro B cell response to type 4 pneumococcal polysaccharide
Immunol Lett
(1992) - et al.
Anti-pneumococcal antibody response in normal subjects: a meta-analysis
J Allergy Clin Immunol
(1996) - et al.
Spectrum of IgG2 subclass deficiency in children with recurrent infections: prospective study
J Pediatr
(1986) - et al.
Bronchiectasis in patients with alpha-1-antitrypsin deficiency: a rare occurrence?
Chest
(1993) - et al.
Usefulness of a national registry of alpha-1-antitrypsin deficiency: the Spanish experience
Respir Med
(1998) - et al.
Alpha 1-antitrypsin phenotypes including M subtypes in pulmonary disease associated with rheumatoid arthritis and systemic sclerosis
Arthritis Rheum
(1986) - et al.
Familial hypogammaglobulinemia: genetic linkage with alpha-1-antitrypsin deficiency
Arch Intern Med
(1983)
Variability of pulmonary function in alpha-1-antitrypsin deficiency: clinical correlates
Ann Intern Med
Serial measurements of FEV1over 12 years in a patient with alpha-1-proteinase inhibitor deficiency: influence of augmentation therapy and infections
Respiration
Primary immunodeficiency diseases: report of a WHO Scientific Group
Clin Exp Immunol;
Recurrent sinopulmonary infection and impaired antibody response to bacterial capsular polysaccharide antigen in children with selective IgG-subclass deficiency
N Engl J Med
IgG subclass deficiencies associated with bronchiectasis
Am J Respir Crit Care Med
An immunodeficiency characterized by impaired antibody responses to polysaccharides
N Engl J Med
Cited by (31)
COVID-19 vaccination in patients with α1-antitrypsin deficiency
2021, The Lancet Respiratory MedicineCystic fibrosis and non-cystic fibrosis bronchiectasis (in adults)
2020, Revue des Maladies Respiratoires ActualitesAssociation between α<inf>1</inf>-antitrypsin and bronchiectasis in patients with humoral immunodeficiency receiving gammaglobulin infusions
2018, Annals of Allergy, Asthma and ImmunologyCitation Excerpt :However, to date, no genetic link has been discovered. It is worth mentioning that the 2 subjects in this study with PiZZ had hypogammaglobinemia, did not respond to pneumococcal polysaccharide vaccine,19 and had a mannose-binding lectin deficiency. However, they did not meet the full criteria for CVID.1
Recommendations for aetiological diagnosis of bronchiectasis
2016, Revista Portuguesa de Pneumologia (English Edition)Citation Excerpt :One small case-control study reported a possible increased frequency of the Z allele among patients with bronchiectasis and common variable immunodeficiency.23 Two other studies suggest that immune deficiency may combine with selected AATD phenotypes to elicit clinically significant bronchiectasis.10,24 Parr et al. have demonstrated that 27% of 74 AAT-deficient patients had High-Resolution Computed Tomography (HRCT) evidence of BE.
Bronchiectasis in a diverse US population: Effects of ethnicity on etiology and sputum culture
2012, ChestCitation Excerpt :Three of these patients had abnormal titers to Pneumovax 23 immunization. Reduced serum levels of specific antipneumococcal IgG, IgG1, and IgG2 after immunization was also shown in patients with bronchiectasis and AAT abnormality by Miravitlles et al,28 and the present data suggest that immune deficiency may combine with selected AAT deficiency phenotypes to elicit clinically significant bronchiectasis. The present study examined bronchiectasis in a diverse population.
Is it asthma, is it bronchiectasis...or is it an alpha-1-antitrypsin deficiency?
2011, Archivos de Bronconeumologia
Supported by Fondo de Investigaciones Sanitarias (FIS) grant 93/0482