Selective IgA deficiency, juvenile idiopathic arthritis and anterior uveitis in a Costa Rican child. Coincidental diseases?. Case report and literature review

Abstract

Backgroup: selective IgA deficiency is the most frequent primary immunodeficiency worldwide. Patients are usually asymptomatic. However, those cases with symptoms develop recurrent infections and increased risk of autoimmune and malignant diseases. On the other hand, rheumatic disorders are uncommon during childhood with juvenile idiopathic arthritis as the most common one. Case Presentation: we present the case of a female patient, who developed oligoarticular juvenile idiopathic arthritis at age 7 years. After the diagnosis, she developed acute anterior uveitis. During the initial immunological evaluation, the diagnosis of selective IgA deficiency was confirmed. A work-up for immunodeficiency demonstrated a normal T cell compartment. B cell subpopulations showed normal memory B lymphocytes, absence of transitional B cells, and an increase in the CD21 low unique subset. Conclusions: at the beginning of any rheumatological evaluation, the physician should request immunoglobulins levels, in order to detect possible primary antibodies deficiencies.