[CIS PIDD] Angioedema in CVID

[Seidel Markus]

Dear Kate,
Was there any dentist intervention / permanent braces etc. before the onset?
Thanks, Yours, Markus Seidel

Markus G. Seidel, M.D., Assoc.Prof.
Consultant| Dept.of Pediatric Hematology-Oncology | Univ.Clinics of
Pediatric and Adolescent Medicine | Auenbruggerpl. 34/2 | A-8036 Graz |
Austria | T. 0043 316 385 80215| F. 0043 316 385 13450
Coordinator of the Working Group for Pediatric Immunology of the Austrian
Society of Pediatrics and Adolescent Medicine

-----Ursprüngliche Nachricht-----
Von: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] Im Auftrag von Sullivan, Kathleen
Gesendet: Donnerstag, 16. August 2012 22:56
An: pagid at list.clinimmsoc.org
Betreff: [CIS PIDD] Angioedema in CVID

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

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!
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