[CIS PIDD] Angioedema in CVID

[Riedl, Marc]

Kate -

1) Does he take any medications other than IVIG?
2) Has he been on high-dose antihistamines (4x labeled doses) for a
prolonged period?
3) Given that this "lasts for weeks", has a tissue biopsy been done?

Marc

Marc Riedl, M.D., M.S.
Associate Professor of Medicine
Section Head, Clinical Immunology and Allergy
UCLA - David Geffen School of Medicine
10833 Le Conte Ave, 37-131 CHS
Los Angeles, CA  90095-1680
Tel 310.206.4345  Fax 310.267.009





On 8/16/12 1:56 PM, "Sullivan, Kathleen" <sullivak at mail.med.upenn.edu>
wrote:


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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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