[CIS PIDD] Angioedema in CVID

[Kathleen Sullivan]

Thanks to all who have provided some much needed ideas on this case.

Although he responds to epi, he clearly does not repond to steroids nor Firazyr.  In a way, that is a really puzzling aspect.  I would have thought that if he responded to epinephrine that meant the mechanism was allergy.  BUT, he has had tons of steroids and a trial of Xolair that was not effective.  We didn't give multiple doses of Xolair but there was not even a hint of improvement after one dose.

Regarding the thoughts that this might be SVC, he has not had exactly the right imaging, but a head CT and PET scan were negative.  We have not done angiography but his swelling is not symmetric.

Kate

----- Original Message -----
From: "Charles Kirkpatrick" <Charles.Kirkpatrick at ucdenver.edu>
To: pagid at list.clinimmsoc.org
Sent: Friday, August 17, 2012 11:48:16 AM
Subject: Re: [CIS PIDD] Angioedema in CVID

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Given that IgG contains a lot of antibodies against HLA molecules, this could be the cause of his attacks if there is a temporal relationship between attacks of angioedema and administration of IgG.  I would check for evidence of complement activation (C4a, MAC, C1Q, etc) and complement consumption.  Can you prevent the attacks by pretreatment with an anti-bradykinin pathway drug?

Chuck Kirkpatrick, M.D.

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Sullivan, Kathleen
Sent: Thursday, August 16, 2012 2:56 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS PIDD] Angioedema in CVID

The CIS-PIDD listserv is supported by:
Clinical Immunology Society - The science & practice of human immunology

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E: info at clinimmsoc.org

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Hi all-
Appreciate any thoughts.

15 year old boy who has CVID-specific antibody deficiency.  He doesn't quite fulfill the diagnosis for CVID (low IgG only and poor vaccine responses).  He was diagnosed about 4 years ago and has been on immunoglobulin replacement ever since.  He did quite well until a year ago when he started developing angioedema.  The episodes are ALWAYS head and neck.  He does have an allergic disposition and has had a few episodes of hives in his life and has required an inhaler for asthma since a young age but his allergies have not been a big deal.

This angioedema is a HUGE deal.  Starting a year ago, he has had angioedema over half the days and has been in the ICU multiple times.

Here is what we know:

Labs:

Normal functional C1 esterase inhibitor
Normal serial tryptases
Normal eosinophil count
Normal thyroid studies

Clinically:

The episodes are unpredictable and can be a little blip or a massive swelling that lasts for weeks.

The do NOT respond to steroids, Firazyr, high dose IVIG

The only thing the episodes respond to is epinephrine and he has had massive amounts of epi.  Although he responds, the swelling recurs until it has run its course.

Any thoughts????? We are running out of options.

Kate

Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



-- 
Kathleen E. Sullivan, MD PhD
Chief, Division of Allergy and Immunology
Wallace Chair of Pediatrics
Director, Immunology Clinic
The Children's Hospital of Philadelphia
Phone: 215-590-1697
Fax: 267-426-0363