Descriptive Study of 123 Symptomatic Patients with IgA Deficiency, a Retrospective Case Series Study

Abstract

Background: IgA deficiency (IgAD) is the most common primary immunodeficiency, which is caused by a defect in IgA antibody production. Most of the patients are asymptomatic. However, patients can present various manifestations. This study was designed to assess the clinical and laboratory manifestations of symptomatic patients with IgA deficiency.
 Method: A group of 123 patients with IgA deficiency referred from all over the country to the national immunodeficiency registration center were entered and followed in this study. The data including demographic characteristics, clinical manifestations and laboratory findings recorded at the registry and also the follow-up visits were extracted.
 Results: The mean age of studied patients was 17.1 years old. Regarding gender, 45 patients (36.5%) were female. The most common clinical presentations included upper respiratory tract infections in 22 (17.9%), enteropathy in 9 (7.9%), allergic rhinitis in 11 (8.9%), sepsis in 4 (3.3%) patients. Four cases of leukopenia with white blood cell (WBC) <4,000/μl and 21 cases of leukocytosis with WBC> 10,000/μl were observed based on the laboratory results. Moreover, IgG2 and IgG4 in 2 and 11 patients were less than normal rate for their age, respectively.
 Conclusion: Although IgA deficient patients are almost always asymptomatic, clinical manifestations such as recurrent sinopulmonary infections, multiple autoimmune diseases, allergic respiratory and skin disorders, gastrointestinal diseases, and rarely severe life-threatening infections could occur.