%0 Journal Article %T Reply %A Allen P. Kaplan %A Anete S. Grumach %A Anna Feldweg %A Chiara Suffritti %A Dana Vuzman %A Henriette Farkas %A Konrad Bork %A Kusumam Joseph %A Lakiea Wright %A Lilian Varga %A Marco Cicardi %A Natasha Frank %A Rohit Sharma %A Rosemeire N. Constantino-Silva %J Journal of Allergy and Clinical Immunology %D 2017 %R 10.1016/j.jaci.2016.12.983 %X The comments by Marlu et£¿al1 regarding our article on plasminogen activator inhibitor 2 (PAI-2) deficiency in patients with hereditary angioedema with normal C1 inhibitor (HAE-N) is timely and interesting. The very low levels observed in 22 of 23 untreated patients with HAE-N (with or without a factor XII mutation, 4.5£¿¡À£¿5) were similar to the levels in 11 patients reported in their commentary, but the PAI-2 mean values in normal controls (54£¿¡À£¿24) and patients with HAE type I (84£¿¡À£¿52) were over 10 times higher. %U https://www.jacionline.org/article/S0091-6749(17)30234-8/abstract