Alpha-1-antitrypsin levels and clinical symptoms in forty-eight children with selective IgA deficiency

Abstract

Previous studies in adult patients with selective IgA deficiency have pointed towards a possible connection between combined IgA and alpha-1-antitrypsin deficiency and the development of bronchiectasis. Thus, investigations were conducted of serum alpha-1-antitrypsin levels and clinical symptoms in 48 children with selective IgA deficiency. However, alpha-1-antitrypsin deficiency was not found in this group of patients. Furthermore, radiographic signs of recurrent pneumonia restricted to certain fields of the lungs, suggestive of bronchiectasia, were not observed in the patients. Thus, although a highly selected group of children with selective IgA deficiency was studied, a connection between IgA deficiency and alpha-1-antitrypsin deficiency was not obvious.

Clinical manifestation for immunoglobulin A deficiency: a systematic review and meta-analysis

“…The prevalence of at least one autoimmunity in SIgAD patients was assessed in 18 studies and ranged from 4.2% by Østergaard P.A. from Denmark (95% CI: 0.012–0.140) [ 17 ] to 39.2% by Kanoh T., et al from Japan (95% CI: 0.168–0.687) [ 18 ]. The pooled prevalence of autoimmunity in the 1,596 study populations was 22.0% (95% CI: 18.0, 26.4%; I2 = 71.8%) ( Fig.…”