News From The Center – October 2015
Greetings from the US HAEA Angioedema Center
It was wonderful to see all of you at the ‘Patient Summit’ in Denver. Hopefully you were able to attend the keynote lectures from Drs Zuraw and Reidl. I want to thank you as well for your ongoing interest in participating in research projects. Dr Banerji and I presented some of the data we published from work done at the last Patient Summit in 2013. I also had a chance to share some very recent data from our current study on the role of stress in HAE and risk of PTSD. Iris Otani has already submitted an abstract to present the findings from the ‘HAE Experience in the Emergency Room’ survey at the upcoming West Coast Allergy, Asthma and Immunology meeting in January. It is really through your efforts that we will make continued progress towards ‘solving the swelling’.
At the time of our last ‘News from the Center’. Drs Riedl, Zuraw and I were on our way to Washington DC where we hosted the second international consensus conference on HAE nl C1 inhibitor with the HAEA. It was an excellent and productive meeting. Scientists from around the world presented their most recent data on this topic. Dr Renne from Stockholm demonstrated the importance of the factor XII mutation and its defective glycosylation resulting in increased kinin mediated vascular permeability (swelling). It appears that the mutation causes the protein to be more ‘activateable’ than its normal counterpart. Drs Joseph and Kaplan from Charleston presented data on the role of estrogen enhancing activation of kallikrein activity and a possible marker for HAEnlC1inh—low levels of PAI-2. Dr Maas from the Netherlands discussed the role of plasmin in the disease. Dr Grumach from Brazil and Dr Caballero from Spain shared data on their large cohort of patients and thoughts on diagnosis, treatment and classification. Dr Bork from Germany presented an interesting case of Tamoxifen triggering attacks of swelling. There were several engaging discussions with other international colleagues and members of our HAEA Medical Advisory Board. These were just some of the highlights. We hope to write up the presentations to provide an updated consensus document on our current understanding of the pathogenesis, diagnosis and treatment of HAE nl C1 inhibitor.
Here at the Angioedema Center we are continuing to work on our basic research into disease mechanisms for HAE type I, II and nl C1 inh. We look forward to sharing more information soon on our HAE & PTSD study—thank you to the many patients who signed up at the Summit. We continue with an active clinical trial program as well. Ongoing is the study on subcutaneous C1 inhibitor with CSL Berhing, and the oral kallikrein inhibitor from BioCryst. We will soon be starting a study on the fully human monoclonal against plasma kallikrein from Dyax. It is exciting to see all of the ongoing progress in the field.
We look forward to sharing more ‘News from the Center’ with the angioedema community in the months to come. We welcome your feedback. We feel very fortunate to have the support of our staff at the Center, the HAEA and all of you.