[CIS PIDD] Angioedema in CVID

[Berger, Melvin]

Kate- does he have any sort of chronic pharyngitis, recurrent step, enlarged chronially tonsils or active EBV infection ? 
 
To the extent that vasodilatation (as opposed to increased capillary permeability contribute to angioedema) epi should help as a vasoconstrictor- I don't think it is in any way specific for allergy. The type 2 bradykinnin receptor tends to increase permeability without vasodilation, which is why HAE atytacks are usually not accompanied by reddness or warmth, and why Epi doesn't necessarily work in HAE.Complement anaphylotoxins have multiple mechanisms of action- directly on endothelial cells and glands as well as on mast cells. Their effects should not be blocked by Xolair but to some extent may be inhibited by antihistamines and epi.
 
Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Routes, John
Sent: Sun 8/19/2012 3:57 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Angioedema in CVID



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Kate
Despite his lack of response to icatibant, I would try prophylaxis with
c1q-inhibitor ----could also try early treatment with c1q-inhibitor if he
does not have frequent enough episodes to warrant prophylaxis.
Jack

John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Co-Director, Clinical and Translational Science Institute of Southeast WI
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-266-6840 (Administrative Assistant)
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Email: jroutes at mcw.edu





> From: Kate Sullivan <sullivak at mail.med.upenn.edu>

> Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Date: Fri, 17 Aug 2012 12:00:50 -0400

> To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>

> Subject: Re: [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!

> 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

>

> 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!

> 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

>

> 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

>

>

>

> --

> 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

>








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