[CIS PIDD] [cis-pidd] Renal Disease and CVID - IVIG replacement on haemodialysis (HD)

Tue Apr 26 02:08:40 EDT 2016

Also, BTW

I would like to know if there is any urine excretion left, protein loss also due to nephrosis? Even IgG can be quantitated from urine, though not many labs do it routinely.

Mikko

oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE), HUS

Mikko Seppänen, MD, PhD, Associate professor
Specialist in Internal Medicine and Infectious Diseases
Head, Rare Disease Center, Helsinki University Hospital (HUH)
Children's Hospital, P.O.Box 280
FI-00029 HUS
FINLAND
&
Senior Consultant (PIDD)
Adult Immunodeficiency Unit
Inflammation Center, HUH

phone +358 9 47180201
GSM +358 50 4279606
fax +358 9 47174703

Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 26. huhtikuuta 2016 1:09
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net>
Aihe: Re: [cis-pidd] Renal Disease and CVID - IVIG replacement on haemodialysis (HD)

Thanks so much to Kate and Nacho for your responses and very helpful comments.

On Kate's first issue of how best to achieve a trough serum IgG level that is required to keep her free of most infections (ideally targeted at 8-10 g/L), her highest level of serum IgG was in July 2014 - 7.14 g/L. Since then she has been on SC Beriglobin via a "push" method of delivery, & her serum IgG has been around 4-5 g/L. She has recently been administering 16 ml of Beriglobin-P 5 days a week (12,8 grams of IgG weekly) which is a higher dose than previously, and the latest serum IgG on 21 April was certainly improved at 6.49 g/L. We just need to exclude intestinal losses as Nacho suggested, and I will  arrange an alpha-1 anti-trypsin clearance. Not sure how I can address the issue of elevated catabolism of IgG in a patient on weekly SC.

I will also look into how we can arrange sequencing to diagnose a "CVID-like" monogenic disorder.

Thanks again.

Regards,

Stan

From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Thursday 21 April 2016 at 12:31 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: Re: [cis-pidd] Renal Disease and CVID - IVIG replacement on haemodialysis (HD)

I think there are two components to this story.  The first is how to best achieve a trough level that is sufficient to prevent most infections.  The second is to determine if this CVID patient has a monogenic disorder that you might treat completely differently.

In general, when we see loss or high catabolism of IgG, it may not be appropriate to target the usual trough level.  Always a goal, but the side effects/cost/inconvenience may not allow it.  I think more frequent subQ infusions often will allow you to get higher levels but in some patients, the losses are simply too overwhelming and it is like pouring the IVIG down the sink. That doesn't seem to be case for your patient and I think tweaking the immunoglobulin regimen may help you get closer to goal troughs.

To come back to the second question- when there is a lot of autoimmune disease, I think of monogenic disorders that have a CVID phenotype. Sometimes, weirdly, immune suppression can help the trough by decreasing catabolism.  I'm not sure how easy it is for you to get access for sequencing but a completely different approach would be to try to determine if this is a PI3 kinase defect or other monogenic form and then you might try to target that pathway.

Kate
Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363
------------------------------------------------------------------------------------------------------------------------
Dear Stan,
I would definitely go for SCIG (and be prepared to combine weekly scig + monthly ivig if the desired levels are not achieved). I would recheck intestinal losses are not present.
Hope this helps
Nacho

Luis I. Gonzalez-Granado. MD.
Immunodeficiencies Unit.
Hospital 12 de octubre.
Research Institute Hospital 12 octubre (i+12)
Av. Cordoba S/N. 28041. Madrid. Spain
Tel. 0034606732959 /  0034913908569  /  Fax 0034913908772<tel:0034934893039>
luisignacio.gonzalez at salud.madrid.org<mailto:luisignacio.hdoc at salud.madrid.org>
ORCID ID:  orcid.org/0000-0001-6917-8980<http://orcid.org/0000-0001-6917-8980>
Researcher ID: B-9257-2009
ResearchGate:https://www.researchgate.net/profile/Luis_Gonzalez-Granado
LinkedIn:  https://es.linkedin.com/in/nachgonzalez

On Apr 21, 2016, at 6:02 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Dear colleagues,

I posted a query a year ago concerning a  36 year-old lady with CVID who had several complications including bronchiectasis, massive splenomegaly and hypersplenism requiring splenectomy, and  liver disease with nodular regenerative hyperplasia. While receiving 3% sucrose IVIG replacement she developed renal impairment. Renal biopsy showed interstitial nephritis and she was switched to SC Beriglobin by "push" administration. Unfortunately her renal failure progressed and she has required HD. The problem is that we have not been able to get her serum IgG at the required trough level of 7-8 g/L (it's usually only 5 g/L) and she has had continued sinus infections, recently complicated by staphylococcal infection of the AV graft.

My questions are:

1.       Since she is on HD twice weekly, would there be any concern about reverting to 3% sucrose IVIG preparation which would permit higher doses of Ig replacement,  and a better chance of reaching required serum IgG level? (I guess the alternative would be to administer SC Ig via a pump to try for optimal levels).

2.       Are there any other considerations regarding the kinetics of IVIG in a patient on HD, any factors related to frequency of administration, catabolism of the product, etc? Presumably we would simple monitor the serum IgG weekly to determine dose and frequency of administration.

3.       Any other advice?

I would appreciate any input, suggestions, advice.

Thanks & Regards,

Stan

Stanley Ress
Emeritus Associate Professor of Medicine, UCT
Specialist physician & Clinical Immunologist
UCT Private Academic hospital,
Anzio Road, Observatory,
Cape Town, 7925
South Africa
TEL:INTERN<tel:INTERN>. + 2721-4421966 or 4421816
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