[CIS-PAGID] Peripheral neuropathy in CVID

[Richard Wasserman]

I agree with the points Jason makes. I don't understand the pathogenesis of
most peripheral neuropathies and I suspect its possible that the proline is
playing a role. I have been involved with a teenager with stiff person
syndrome who experienced a dramatic deterioration when given a glycine
containing IVIG (I don't recall which one) for CVID that predated the SPS.
She was found to have an autoantibody to an enzyme that was inhibited by
glycine. This may or may not be relevant to the question of peripheral
neuropathy and proline.
Richard Wasserman

On Wed, Apr 18, 2012 at 9:09 PM, Jason Raasch, MD <raas0027 at umn.edu> wrote:


>

>

> The dichotomy of our reference to immunoglobulin therapy is interesting:

>  to third-party payors, pharmacies, etc. we preach the importance of having

> a choice for our patients as there are clear differences among products.

>  Yet in our academic discussions and communications we simply refer to it

> as "IVIG" or "SQ" (as if mentioning a brand is irrelevant or a delicate

> subject).

>

> In patients who have apparent reactions to immunoglobulin therapy I think

> we must discuss the specific preparations, not just the route.

>

> Howard, it would be of interest to know how long the first patient you

> mentioned was on Hizentra before the onset of neuropathy symptoms and if

> this patient had been transitioned to it from IVIG (and which one!).  Same

> thing for the out-of-town patient referred to.

>

> Agree with the question Richard poses with regard to Privigen, as a common

> denominator between it and Hizentra is proline.  Sweden and UK data may

> suggest SQ vs IV (ROUTE!) is not the issue, but what about specific

> product?  I admit I cannot quote specifics of these studies - I'll get

> back to you on that after I review.

>

> Perhaps related to this is Joe's patient with presumed mitochondrial

> disorder.  The key enzymes required for proline metabolism are proline

> dehydrogenase and P5C, both located in the mitochondria.  Could the

> neuropathy be related to proline toxicity (from Hizentra) secondary to the

> mitochondrial disorder (dysregulated proline metabolism)?  Yes, this is a

> stretch but I again argue that these are the types of questions we need to

> ask.

>

> Agree with Marc  that in that these patients  with apparent difficulty

> tolerating Hizentra, it IS important to try to switch to the other SQ

> products.  I have two patients who tolerated Vivaglobin well.  Flu-like

> symptoms with hizentra (that admittedly I did not believe for a while).

>  Tried SQ Gamunex with similar symptoms.  Transitioned to SQ Gammagard and

> both have tolerated as well as Vivaglobin.  Switched BACK to Hizentra

> (these were very willing patients) and flu-like symptoms appeared again.

>

> Kate's concern is valid and a call to discuss and study not just SQ vs

> IVIG (ROUTE!) but also Gammagard vs Privigen vs Gammaplex vs [all others].

>  Yes, this would be difficult - but what hasn't been in clinical immunology?

>

>

>

> -Jason

>

>

> Jason Raasch, MD

>

> Midwest Immunology Clinic

> 15700 37th Ave N

> Ste 110

> Plymouth, MN 55446

>

> Phone: (763) 577-0008

> FAX:     (763) 577-0192

>

>

>

> On Apr 18, 2012, at 14:31 PM, Howard Lederman wrote:

>

> 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****

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>

>

>



-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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