HAE with normal C1 inhibitor (HAE-nl-C1INH) – Twitter summary from the 2019 WSAAI meeting

Dr Zuraw talked about HAE with normal C1 inhibitor.

Women are more commonly affected by HAE with normal C1 than men. HAE with normal C1 can skip generations unlike normal HAE, mainly because it has low penetrance.

UCSD only has two families with Factor XII in their registry, showing just how rare that is. Impact of Factor XII on HAE-nl-C1INH: https://twitter.com/MatthewBowdish/status/1087834677287706625

HAE classification is getting more complicated – 4 types of HAE-nl-C1INH, and counting: https://twitter.com/MatthewBowdish/status/1087837543536578562

Current classification of Angioedema: https://twitter.com/MatthewBowdish/status/1087838090775777280

You cannot separate HAW-nl-C1INH from non-histaminergic bradykinin angioedema except by family history +/- genes.

Clinical findings that help distinguish between HAE and HAE-nl-C1INH: https://twitter.com/MatthewBowdish/status/1087839803570188288

Differentiating HAE normal and deficient C1 inh – note facial swelling: https://twitter.com/RayFirszt/status/1087846754320449537

Current treatment approach for HAE-nl-c1INH: https://twitter.com/MatthewBowdish/status/1087842869988052993

Treatment for HAE n C1inh: progestin and TXA are effective. C1inh can help during active attacks in HAE n C1inh because of deficiency that occurs during angioedema. C1inh cannot be used for prophylaxis. Lanadelumab is a monoclonal Ab against kallikrein. It should work in HAE w normal C1inh.

Phase 2 trial: Factor XIIa monoclonal ab – CSL312. It should work since kallikrein activation goes through factor XII.

Biomarker development for angioedema: measuring kallikrein activity can distinguish between histaminergic and non histaminergic angioedema. 93% PPV and 86% NPV. Patient can’t be on treatment for 1 week prior to test.

Biomarkers for HAE using kallikrein activity: https://twitter.com/RayFirszt/status/1087846989918699520

This is a Twitter summary from the 2019 WSAAI meeting. This summary was compiled from the tweets posted by Matthew Bowdish @MatthewBowdish and Ray Firszt @RayFirszt, who attended the 2019 Western Society of Allergy, Asthma and Immunology (WSAAI) meeting. The tweets were labeled #WSAAI. The text was edited by me.

Different causes of bradykinin-related angioedema. Click here to enlarge the image.



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