Importancia del déficit selectivo de inmunoglobulina A / Importance of selective immunoglobulin A deficiency

La deficiencia selectiva de inmunoglobulina A (IgA) es la inmunodeficiencia primaria más común. Se conoce muy poco sobre los mecanismos etiopatogénicos que conducen a esta enfermedad. Se estima que la incidencia de la enfermedad en España es de 1:163. Presentamos 3 casos diagnosticados en atención primaria. La mayoría de las personas con déficit selectivo de IgA suelen estar asintomáticas y se diagnostican por casualidad; sin embargo, deberíamos tener presentes a los pacientes con infecciones de vías respiratorias recurrentes, gastrointestinales, alérgicas y enfermedades autoinmunes que pueden estar asociadas a esta afección. A pesar de que no existe tratamiento en el momento actual, su importancia radica en las asociaciones con diferentes enfermedades como la enfermedad celíaca o púrpura trombocitopénica idiopática. Por otra parte, es importante conocer el riesgo de anafilaxia tras realizar transfusiones y su posible progresión a una inmunodeficiencia común variable. Es importante conocer la correcta vacunación en esta enfermedad (AU)

Selective IgA deficiency is the most common primary immunodeficiency. Little is known about the pathogenetic mechanisms leading to this disease. It is estimated that the incidence of this disease in Spain is 1:163. We report 3 cases diagnosed in Primary Care. Most people with selective IgA deficiency are usually asymptomatic and are diagnosed by chance, but patients with recurrent respiratory infections; gastrointestinal, allergic and autoimmune diseases can be associated with this disease. Although there is no treatment at present, its importance lies in the association with different diseases, such as coeliac disease or idiopathic thrombocytopenic purpura. Another important feature is the risk of anaphylaxis after transfusion, and the possible progression to Common Variable Immunodeficiency. It is important to know the vaccines that we can use due to the risk of disease (AU)

[Importance of selective immunoglobulin A deficiency]. / Importancia del déficit selectivo de inmunoglobulina A.

Selective IgA deficiency is the most common primary immunodeficiency. Little is known about the pathogenetic mechanisms leading to this disease. It is estimated that the incidence of this disease in Spain is 1:163. We report 3 cases diagnosed in Primary Care. Most people with selective IgA deficiency are usually asymptomatic and are diagnosed by chance, but patients with recurrent respiratory infections; gastrointestinal, allergic and autoimmune diseases can be associated with this disease. Although there is no treatment at present, its importance lies in the association with different diseases, such as coeliac disease or idiopathic thrombocytopenic purpura. Another important feature is the risk of anaphylaxis after transfusion, and the possible progression to Common Variable Immunodeficiency. It is important to know the vaccines that we can use due to the risk of disease.