Autoimmune diseases and autoantibodies in pediatric patients and their first-degree relatives with immunoglobulin A deficiency

Author(s)

Kristine Fahl ^[1] ; Clovis A. Silva ; Antonio C. Pastorino ^[3] ; Magda Carneiro-Sampaio ^[4] ; Cristina M.A. Jacob ^[5]

Affiliation

^[1] Universidade de São Paulo. Faculdade de Medicina. Departamento de Pediatria - Brasil

^[3] Universidade de São Paulo. Faculdade de Medicina. Departamento de Pediatria - Brasil

^[4] Universidade de São Paulo. Faculdade de Medicina. Departamento de Pediatria - Brasil

^[5] Universidade de São Paulo. Faculdade de Medicina. Departamento de Pediatria - Brasil

• Document type: Journal article
• Source: REVISTA BRASILEIRA DE REUMATOLOGIA; v. 55, n. 3, p. 197-202, 2015-06-00.

Abstract

Introduction: Clinical manifestations of Immunoglobulin A Deficiency (IgAD) include recur-rent infections, atopy and autoimmune diseases. However, to our knowledge, theconcomitant evaluations of autoimmune diseases and auto antibodies in a cohort of IgADpatients with current age >10 years and their relatives have not been assessed. Objectives: To evaluate autoimmune diseases and the presence of auto antibodies in IgADpatients and their first-degree relatives. Methods: A cross-sectional study was performed in 34 IgAD patients (current age >10years) and their first-degree relatives. All of them were followed at a tertiary Brazilianprimary immunodeficiency center: 27 children/adolescents and 7 of their first-degree rela-tives with a late diagnosis of IgAD. Autoimmune diseases and autoantibodies (antinuclearantibodies, rheumatoid factor, and anti-thyroglobulin, anti-thyroperoxidase and IgA classanti-endomysial antibodies) were also assessed. Results: Autoimmune diseases (n = 14) and/or autoantibodies (n = 10, four of them with iso-lated autoantibodies) were observed in 18/34 (53%) of the patients and their relatives. Themost common autoimmune diseases found were thyroiditis (18%), chronic arthritis (12%)and celiac disease (6%). The most frequent autoantibodies were antinuclear antibodies(2%), anti-thyroglobulin and/or anti-thyroperoxidase (24%). No significant differences wereobserved in the female gender, age at diagnosis and current age in IgAD patients with andwithout autoimmune diseases and/or presence of auto antibodies (p > 0.05). The frequen-cies of primary immunodeficiencies in family, autoimmunity in family, atopy and recurrentinfections were similar in both groups (p > 0.05). Conclusion: Autoimmune diseases and auto antibodies were observed in IgAD patients dur-ing follow-up, reinforcing the necessity of a rigorous and continuous follow-up duringadolescence and adulthood. (AU)

• FAPESP's process: 08/58238-4 - Autoimmunity in children: investigation of the molecular and cellular bases of early onset of autoimmunity
• Grantee: Magda Maria Sales Carneiro-Sampaio
• Support Opportunities: Research Projects - Thematic Grants