Introduction
Selective IgA deficiency is the most common primary selective immunodeficiency, defined as serum IgA less than 4 years old, with normal IgM and IgG values. Is has a variable clinical presentation, with prior exclusion of other causes of hypogammaglobulinemia or T cell defects.
Cases
Female, 20 years old with idiopathic thrombocytopenic purpura, recurrent respiratory infections, asthma and chronic diarrhea. She has had 3 measurements of IgA < 3 mg/dl, IgG and IgM levels are normal. Under surveillance. Male, 41 years old, with idiopathic thrombocytopenic purpura, hypoparathyroidism, nephrotic syndrome, and 8-10 episodes of respiratory infections. First IgA measurement 0.10 mg/dl, second and third measurements < 7 mg/dl, IgG and IgM levels are normal. Under surveillance. Female, 52 years old, with gastric neuroendocrine tumor type 1, pernicious anemia, chronic gastritis and primary hypothyroidism. First IgA measurement 1.20 mg/dl, second and third measurements < 4 mg/dl. IgG and IgM levels are normal. Under surveillance. Female, 40 years old, with subclinical hypothyroidism, asthma, chronic urticaria and respiratory infections, requiring hospitalization for pneumonia. She has two measurements of IgA < 7 mg/dl, IgG and IgM levels are normal. Lost to follow-up. Male, 13 year old, developed viral hepatitis, later developed heart failure with an ejection fraction of 30%, and was diagnosed with dilated cardiomyopathy. IgA was measured on 3 occasions and was < 7 mg/dl, IgG and IgM levels are normal. Deceased. Discussion Selective IgA deficiency has a variable clinical presentation, ranging from asymptomatic patients to those with infections (40% respiratory), autoimmunity (25-31%), endocrinopathies and allergies (84%). Notably, autoimmune conditions and recurrent respiratory infections are prominent among our patients, with no gender predominance, and diagnosis occurring in adulthood in 80% of cases. Of these, 3 are under surveillance, one was lost to follow-up, and the last one deceased.