PO-1016 Phenotype Variety In Patients With Selective Iga Deficiency

Abstract

<h3>Background</h3> Selective IgA deficiency (SIgAD) is the most frequent primary antibody deficiency. <h3>Aims</h3> Authors emphasise the phenotype diversity among SIgAD patients included in our clinic casuistry. <h3>Methods</h3> Authors analysed clinical and biochemical features of patients that fulfilled SIgAD diagnosis criteria. They’ve been evaluated concerning previous respiratory and gastro-intestinal diseases severity (necessity for intravenous antibiotics and/or intensive care measures). Serology investigations performed: complete blood count and evaluations for immunoglobulin isotype, IgG subclass, total IgE, specific IgE for cow’s milk precipitins. Autoimmune status estimation included antibodies against: red blood cells, basement membrane, smooth muscle, thyroglobulin, thyroid microsomal antigens, transglutaminase IgG/endomysium and nuclear proteins. Authors accomplished microbiological tests for patients with sinopulmonary infections and analysed stools in order to exclude Giardia/ Cryptococcus infections. Susceptibility for brochiectasis justified chest X-ray. <h3>Results</h3> Among 8 SIgAD patients followed in our clinic, 3 patients were considered as asymptomatic. The others were diagnosed with: recurrent knee arthritis in context of Epstein-Barr virus reactivation confirmed by quantitative PCR for DNA-EBV in serum (1 patient), celiac disease (2 patients), giardiasis (2 patients), asthma and allergic rhinitis (1 patient), cow milk allergy (1 patient). Sinopulmonary infections/ purulent otitis were identified for all symptomatic cases. One patient was diagnosed with tonsil phlegmon. No immunological thrombocytopenic purpura or autoimmune hemolytic anaemia were confirmed. <h3>Conclusions</h3> 1. In spite of small number of SIgAD patients, authors revealed a quite large range of clinical manifestations: from asymptomatic status to recurrent arthritis due to EBV reactivations; 2. Three patients presented more than 1 clinical feature.