Abstract
Selective IgA deficiency (SIgAD) is the most prevalent immunodeficiency worldwide, progressing to common variable immunodeficiency only in few reported cases. We report the case of a Spanish female aged 22 and diagnosed of selective IgA deficiency, a long history of bronchitis, several episodes of pneumonia, bilateral bronchiectasis, normal IgG, IgM, IgG subclasses, and detectable pre-vaccination IgG antibodies against tetanus toxoid and Streptococcus pneumoniae. She was evaluated in our clinic in order to rule out common variable immunodeficiency. We observed good antibody response to tetanus toxoid, absence of circulating switched memory B cells, decreased response to pneumococcal polysaccharide antigens and a lack of response to Salmonella typhi vaccine. Most SIgAD patients presents with upper respiratory tract infections or mild diarrhea. Those with lower tract infections, pneumonia or untreatable diarrhea should follow B-cell subpopulations’ study and antibody response to vaccines. Absence of response to Salmonella typhi vaccine allowed us to expose the defective antibody production.
References
International Union of Immunological Societies. Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. Clin Exp Immunol 1999;118:1-28. Disponible en: http://onlinelibrary.wiley.com/doi/10.1046/j.1365-2249.1999.00109.x/abstract;jsessionid=F63DD1E9DA8898608BF758C557F6B299.f01t04
Aghamohammadi A, Mohammadi J, Parvaneh N, et al. Progression of selective IgA deficiency to common variable immunodeficiency. Int Arch Allergy Immunol 2008;147:87-92. Disponible en: http://www.karger.com/Article/Full-Text/135694
Orange JS, et al. Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol 2012;130:1-24. Disponible en: http://www.jacionline.org/article/S0091-6749(12)01103-7/abstract
Ferry BL, Misbah SA, Stephens P, et al. Development of an anti-Salmonella typhi Vi ELISA: assessment of immunocompetence in healthy donors. Clin Exp Immunol 2004;136:297-303. Disponible en: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2249.2004.02439.x/abstract
Sánchez-Ramón S, de Gracia J, García Alonso AM, et al. Prospective study (EMPATHY) of the antibody response against S. Typhi for the evaluation of anti-polysaccharide antibody production deficiency. Clin Exp Immunol 2013;174:27-55. Disponible en: Special Issue: Abstracts of the UK Primary Immunodeficiency Meeting 2013, Liverpool, UK. http://onlinelibrary.wiley.com/doi/10.1111/cei.12218/full
Stephan Ehl, et al. New clinical diagnosis criteria for the ESID Registry 2014. Disponible en: http://esid.org/Working-Parties/Registry/Diagnosis-criteria

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