[CIS-PAGID] Gleich Syndrome

[Seppänen Mikko]

Dear Joe,

My pt is just a little eccentric and fussy when not on steroids, nothing to suggest an actual psychiatric or neuropsychologic dg. She responded only to Prednisolone >40mg/d but was controlled/quiescent while on IVIg, nowadays she has Predn available but due to mild symptoms, has not used them in >1 year. And of course visiting ERs while the disease has no name and is under no control can leave its marks in one's psyche.

It is certainly easier to try IVIg in Finland, though at times (different pts) I wonder how beneficial is that either... Have over the years stopped IVIg treatments worth more than 10x the sum of my predicted paychecks before retirement, easily.... and seen a fair share of deleterious effects as well, if that is of any comfort?

m

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Lähettäjä: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] käyttäjän Church, Joseph [JChurch at chla.usc.edu] puolesta
Lähetetty: 4. huhtikuuta 2012 17:18
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: Re: [CIS-PAGID] Gleich Syndrome

Thank you, Mikko.

My patient also has some psych issues.  I wonder if it is part of the syndrome?

He does respond well to steroids the few times we had to go that route and I probably couldn't get IVIG paid for here in the U.S. even if I wanted to try it.

Joe Church



From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Seppänen Mikko
Sent: Tuesday, April 03, 2012 10:52 PM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [CIS-PAGID] Gleich Syndrome

Dear Joe

I have been following a single Gleich’s (a female, age 40+): when her episodic angioedema was severely symptomatic and no therapy seemed to work well (and corticosteroids caused considerable psychiatric side effects among others), based on a single positive case report (and wondering whether a Th2 immunostimulant – a.o. IL-4, IL-33 - called IVig actually could work in angioedema….) I put her on IVIg trial for 1 year. After that – right after the IVIg was commenced-  her symptoms subsided, after stopping IVIg her symptoms have been a lot less severe (???). I used only 0.5 g/kg/month, every 3 weeks.

In my mind, there is a considerable chance that both these cases would have had less symptoms anyway due to natural fluctuations in the disease course and that there actually is no real treatment effect, and I would love to hear if anyone has had any negative treatment experiences with IVIg….

Like infrequently reported in Gleich’s, she has monoclonal T cells in her blood at a very low level, no signs of any malignant T cell clone at follow up (yet?), have followed her some 5 years now. As far as I know, she is the only Gleich’s in Finland, Gleich’s is reportedly very rare, and thus if anyone on this list has more experience with several patients, I would also love to hear…

I do wonder exactly why did it work, and did it truly work, and worry that I might not be giving misleading advice,

Yours,

mikko

__________________________________________________
Mikko Seppänen, MD, PhD
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
EM(E)A Expert, PIDDs and Intravenous Immunoglobulin Therapy

Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945
_________________________________________

Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Church, Joseph
Lähetetty: 3. huhtikuuta 2012 19:46
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: [CIS-PAGID] Gleich Syndrome

Colleagues:

I follow a teen with classic Gleich Syndrome (episodic angioedema, marked weight gain and eosinophilia).

Have there been any recent findings (not on Pub Med or Ovid) on pathogenesis or treatment?

Joe Church
Children's Hospital Los Angeles

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