Premium
Heterozygous α 1 ‐antichymotrypsin deficiency may be associated with cold urticaria
Author(s) -
Lindmark B.,
Wallengren J.
Publication year - 1992
Publication title -
allergy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.363
H-Index - 173
eISSN - 1398-9995
pISSN - 0105-4538
DOI - 10.1111/j.1398-9995.1992.tb00663.x
Subject(s) - serpin , immunology , medicine , c1 inhibitor , hereditary angioedema , tryptase , plasminogen activator inhibitor 1 , serine proteinase inhibitors , antithrombin , angioedema , heterozygote advantage , serine protease , endocrinology , genotype , plasminogen activator , biology , protease , mast cell , enzyme , biochemistry , heparin , gene
Proteins of the serpin family (serine protease inhibitor) control key steps in the inflammatory, coagulation and complement systems. C 1 ‐inhibitor deficiency predisposes to hereditary angioneurotic oedema, and other serpins control proteolytic enzymes that may cause complement activation or the forming of oedema. We investigated whether deficiency of proteins of the serpin family may predispose to cold urticaria and therefore screened 7 male patients with severe cold urticaria for the presence of deficieney alleles of some of the members of the serpin antiprotease family. There were no findings of C 1 ‐inhibitor, α 1 ‐antitrypsin, α 2 ‐antiplasmin, antithrombin III, tissue plasminogen activator inhibitor or thyroxine binding protein deficiency. The prevalence of heterozygous α 1 ‐antichymotrypsin deficieney was significantly higher than expected (prevalence ratio 25.8 (95% confidence interval 6.0‐112), p< 0.0001). This finding is in concert with previous studies that have shown lower mean levels of α 1 ‐antichymotrypsin among patients with cold urticaria and suggests that heterozygous deficiency of this antiprotease, which controls neutrophil eathepsin G and mast cell chymase may predispose to cold urticaria. The present series is, however, small and the results need confirmation in larger materials.