[CIS PIDD] Angioedema in CVID

[Yeşim Yılmaz Demirdağ]

Hi Mike,
I am following a similar case:
He is a 22-year-old man with selective IgA deficiency and environmental
allergies. He started having angioedema symptoms at age 19, he had
peripheral and facial/lip/tongue swellings almost 2-3 times/wk. He was in
the hospital almost every week. On several occasions he had urticarial
lesions as well. Labs were all negative. Symptoms did not respond to
therapeutic dose of H1 and H2 antihistamines, steroids, epi, Kalbitor (he
had allergic reaction to Kalbitor after 3 rd treatment), and high dose
IVIG. He had to quit his job and school and he was in the process of
applying for disability.
Then 1.5 yr ago he started to take Allegra 180 bid, Zyrtec 10 bid, Zantac
300 mg bid, and Singulair 10 mg daily. And surprisingly he did not have any
symptoms for about 6 months when he was on this combination. Moreover, his
symptoms recur within 48 hours when we try to wean one of the H1
antihistamines and when we stop Singulair. He has not been in the hospital
in the past 1.5 yr, he is back to work and school and doing great.
I cannot answer how this combination worked, but it worked. You may want to
try this on your patient. Good luck,
Yesim

Yesim Yilmaz Demirdag, MD
Section of Allergy and Immunology
Department of Pediatrics
Morgantown, WV




On Sat, Jul 7, 2012 at 7:48 PM, Keller, Michael D
<KellerMD at email.chop.edu>wrote:


> Dear colleagues:

>

> We have a very mysterious case of a 16 year old with CVID and severe

> recurrent angioedema, detailed below.  We would be most grateful for any

> thoughts on possible diagnoses or treatments.

>

> Best regards,

>

> Mike Keller

> Children's Hospital of Philadelphia

>

> --------------------

>

> The patient is a 16 yr old with CVID and recurrent facial angioedema

> episodes for the past 8 months.  These episodes occur irregularly, without

> clear trigger, with swelling of the lips and tongue and distressed

> breathing.  They have been associated with hives on the neck in some

> instances.

>

> Three months after onset, he had a several angioedema flare requiring a 7

> week hospitalization, including ICU admission and intubation.

>

> Prior workup of the angioedema:

> -Negative hereditary angioedema testing (normal C1 esterase inhibitor

> function, normal C3/C4)

> -Normal thyroid screening

> -Negative viral studies: Hep B (antigen), Hep C (serologic & pcr), EBV

> (pcr), HIV (Ag/Ab)

> -Negative CIU index, negative ANCA, negative ANA, normal serum ACE

> -Normal tryptase

> -Normal SPEP

> -Negative urine metanephrines

> -Unremarkable PET scan to evaluate for occult infection / malignancy.

>

> He does have multiple environmental and food allergies, but flares occur

> without any allergic triggers (including while inpatient and NPO during his

> most severe episode).  There are no obvious physical triggers for urticaria

> or angioedema, though in a few episodes, he has had a concurrent sinusitis.

>

> With regard to therapies:

> -flares seem to respond to epinephrine, but are steroid refractory

> -antihistamines (allegra, zyrtec, doxepin) have failed to prevent

> episodes.  Diphenhydramine seems to have a minimal subjective impact.

> -During more severe flares, other therapies have been tried without

> impact: FFP, xolair (x 2 doses), icatibant (which seemed to worsen

> swelling).

> -Immunomodulatory (2g/kg) IVIG seemed to improve his most recent flare.

>

> With regard to his CVID history, he was diagnosed at age 13 years, and has

> been maintained on IVIg since that time.   Aside from humoral deficiency,

> he has normal lymphocyte flow cytometry including present switched memory

> B-cells, and normal lymphocyte proliferation to PHA, ConA, and PWM.

>

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