[CIS PIDD] Angioedema in CVID

[Vickery, Brian Patrick]

Hi Kate:

Agree with checking C4 and C1q, but if the swelling responds well and fairly quickly to epinephrine, it is unlikely to be kinin-mediated. Also agree with trying suppression with antihistamines and titrating to effect with high doses as Marc recommends. Obviously if antihistamines work – and it sounds like it wouldn't take long to figure it out – great; but would have a low threshold to use prednisone to gain control especially if there is significant morbidity. One other thought, kind of crazy, but the lone star tick is wandering up into Jersey and Maryland and poised to invade PA if it hasn't already (Figure 4: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3085643/). IgE to alpha gal?

BPV

Brian P. Vickery, MD
Assistant Professor Of Pediatrics
University of North Carolina School of Medicine
Campus Box #7231 | Chapel Hill, NC 27599
919.962.5136 | bvickery at email.unc.edu<mailto:bvickery at email.unc.edu>





On 8/16/12 5:14 PM, "Rafael Firszt" <Rafael.Firszt at hsc.utah.edu<mailto:Rafael.Firszt at hsc.utah.edu>> wrote:

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Hi Kate,

I would rule-out SVC syndrome.   Have you considered a Trach?  When I was at Duke, we had a similar patient with idiopathic angioedema that would not respond to therapy and we had to trach him.
IS C4 normal during attacks, C1q levels (acquired angioedema),  ANA levels?
Is he on any prophylactic medicines?  Antihistamines, Singulair, Attenuated Androgens (some idiopathics still respond for some reason).

Rafael Firszt
University of Utah

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org<mailto: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<mailto:pagid at list.clinimmsoc.org>
Subject: [CIS PIDD] Angioedema in CVID

The CIS-PIDD listserv is supported by:
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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



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