[CIS-PAGID] Peripheral neuropathy in CVID

[Howard Lederman]

I appreciate everyone's comments about this issue.  Again, I am not yet ready to suggest cause and effect, but there already have been enough comments to warrant better data collection.

I will review the chart of my patient (and call the out-of-town physician) to get information about all previous gamma globulin therapy for the two patients that I described in my initial email.  That will include data on brand of gamma globulin, route of administration, dose, and approximate dates of use.  I will create an Excel spreadsheet and suggest that others add their data to mine.  (I can always go back to get lot #s , if necessary.)

I think that I will also d/c Hizentra and switch to Gamunex C, and watch for any change in symptoms. 

Howard
Howard M. Lederman, M.D., Ph.D.
Professor of Pediatrics, Medicine and Pathology
Division of Pediatric Allergy and Immunology
Johns Hopkins Hospital - CMSC 1102
600 N. Wolfe Street
Baltimore, MD 21287-3923
Phone: 410-955-5883
Fax: 410-955-0229
Email: Hlederm1 at jhmi.edu

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From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of John Ziegler [j.ziegler at unsw.edu.au]
Sent: Wednesday, April 18, 2012 10:40 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Peripheral neuropathy in CVID

Thomas

A nephrologist colleague told me an interesting story about his spouse.

In summary she had a progressive motor neuropathy affecting lower limbs- aetiology not clear, possibly autoimmune demyelinating neuropathy.

Started on IVIG as therapeutic trial. Only received one of five planned loading doses because she developed headache, confusion, diplopia, central chest pain,  gross generalised tremor and acute profuse watery diarrhoea. Extensive investigations.

Symptoms persisted for several months, gradually settled. Diarrhoea continued, GI investigations led to diagnosis of lymphocytic colitis, resolution of symptoms with steroids and mesalazine.

In retrospect, central chest pain thought to be first manifestation of coronary artery disease.

No further treatment of motor neuropathy. Clinically stable at approximately 9 months.


John Ziegler

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Thomas Issekutz
Sent: Thursday, 19 April 2012 9:20 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Peripheral neuropathy in CVID


I have also had a 15 year old girl with CVID who did very well on
IVIG. She had been extremely well without any complications for many
years, but wished to try SCIG.  She was switched to sc Vivaglobin but
about six weeks later had some intermittent nausea, occasional
vomiting, and progressive abdominal pain.  Eventually she decreased
her intake and lost substantial weight. She was started back on IVIG
and when scoped had extensive inflammatory bowel disease. She began to
improve over a few weeks after the IVIG and was also placed on an
alimental diet. She regained her weight after about five months. She
is back or a regular diet on IVIG, but continues to have an elevated
CRP. She wants to go back to SCIG. In light of the discussion here,
sounds like that is not a good idea. Thank you for this.

Thomas Issekutz MD
Professor and Head,
Division of Immunology
Department of Pediatrics
IWK Health Centre
Dalhousie University
Halifax, NS CANADA
B3K6R8
Ph 902-470-8933




Quoting "Nelson, Robert P Jr" <ronelson at iupui.edu>:


> Agree wholeheartedly with Kate. I haven't seen this but will look

> more closely thanks to these communications.  Bob

> ________________________________________

> From: pagid-bounces at list.clinimmsoc.org

> [pagid-bounces at list.clinimmsoc.org] on behalf of Sullivan, Kathleen

> [sullivak at mail.med.upenn.edu]

> Sent: Wednesday, April 18, 2012 4:49 PM

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] Peripheral neuropathy in CVID

>

> This discussion has me slightly worried.  I have not seen this but I

> would request of those who have to put their data together and

> report to CSL, write the cases up and perhaps think of querying

> USIDNET to see if there are other cases.

>

> Kate

> On Apr 18, 2012, at 4:29 PM, Riedl, Marc wrote:

>

>> I have an adult CVID patient who developed worsening of

>> pre-existing idiopathic peripheral neuropathy when switching from

>> IVIG to SQ Hizentra.   In addition, her pre-existing

>> gastrointestinal malabsorption (also not well-defined despite

>> extensive GI evaluation) worsened on SQ treatment.  She has

>> recently switched back to IVIG given these developments, but too

>> early to judge whether this move will improve her conditions back

>> to "baseline".    Given the concurrent worsening of these

>> "CVID-associated symptoms" my impression is that there is some

>> beneficial effect of the higher peak levels achieved with IVIG that

>> we're unable to attain with SQ, rather than this being a

>> product-specific side effect.  If a patient is willing (mine was

>> not) would be if interest to switch to a different SQ product to

>> assess effect.

>>

>> Best,

>>

>> 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

>>

>>

>> From: <Church>, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>>

>> Reply-To: pagid listserve

>> <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>

>> To: pagid listserve

>> <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>

>> Subject: Re: [CIS-PAGID] Peripheral neuropathy in CVID

>>

>> I follow a 12yo boy who had a similar experience when switched from

>> IVIG to SC Hizentra.  His peripheral neuropathy (diagnosed by

>> Neurology here) affects an area around the infusion sites and

>> radiates to his scrotum.  These symptoms have persisted despite the

>> switch back to IVIG.

>>

>> The patient also has symptoms consistent with a mild form of

>> mitochondrial disorder and has been diagnosed by our Genetics group

>> as such.  Pain syndromes are often part of the mito picture, so I

>> don't know if his neuropathy is related to mitochondrial

>> dysfunction or the Hizentra infusions, both or neither.

>>

>> Joe Church

>> Children's Hospital Los Angeles

>>

>> From:

>> pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org>

>> [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Howard

>> Lederman

>> Sent: Wednesday, April 18, 2012 12:32 PM

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

>> Subject: [CIS-PAGID] Peripheral neuropathy in CVID

>>

>> I have an adult CVID patient who has developed an idiopathic

>> peripheral neuropathy while on Hizentra.  My first reaction was to

>> think that this was merely a coincidence and not cause and effect.

>> However, yesterday I received a call about an out-of-town patient

>> who had CVID, had recently been switched from IVIG to Hizentra and

>> then  developed parasthesias.

>>

>> Do any of you have patients with similar histories?

>>

>> Howard

>>

>>

>> Howard M. Lederman, M.D., Ph.D.

>> Professor of Pediatrics, Medicine and Pathology Division of

>> Pediatric Allergy and Immunology Johns Hopkins Hospital - CMSC 1102

>> 600 N. Wolfe Street Baltimore, MD 21287-3923

>> Phone: 410-955-5883

>> Fax: 410-955-0229

>> Email: Hlederm1 at jhmi.edu<mailto:Hlederm1 at jhmi.edu>

>>

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>

> 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

>

>

>

>