[CIS PIDD] [cis-pidd] IVIG/ScIg for Complement deficiency?

Tue Apr 14 13:47:06 EDT 2015

As a fellow, I encountered 2 patients from 2 families with S pneumo meningitis that were found to have genetically confirmed complete C2 deficiency.  I know that Patsy Giclas has confirmed the diagnosis in several other patients throughout the country.  Both of our patients had persistent evidence of inflammation following antibiotic treatment that only resolved with initiation of FFP transfusion (there is no available purified or recombinant C2 replacement).  With resolution of the initial infection, we managed with prophylactic antibiotics and s pneumo vaccination (Prevnar < 2yr and Pneumovax >2yr).  Not sure if it is necessary to do other vaccines beyond the usual schedule; although, ensuring adequate Hib titers makes sense.

We surveyed the AAAAI at the time to see how often the problem was being seen and how they were being treated.  It was pretty clear, as all have surmised, there is no consensus in the US on how to treat these patients.  It was also clear that most members of the AAAAI do not see C2 deficiency, and the minority that have seen it have seen it only rarely.  Of those who actually have managed these patients, the diagnosis was considered after invasive bacterial infection in the majority.  I don't know if it is under diagnosed, but the majority of respondents indicated they do not send complement studies on every patient diagnosed with S pneumo invasive infection.  I have pasted a link to the abstract below:
http://www.jacionline.org/article/S0091-6749(12)03226-5/abstract

Jordan Abbott, M.D.
Assistant Professor of Pediatrics
National Jewish Health
1400 Jackson St., J333
Denver, CO 80206

From: "<Prescott Atkinson>", "M.D." <PAtkinson at peds.uab.edu<mailto:PAtkinson at peds.uab.edu>>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Date: Tuesday, April 14, 2015 9:35 AM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: Re: [cis-pidd] IVIG/ScIg for Complement deficiency?

I don't recall ever treating C2 deficiency with IgG infusions - even the one  kid who had recurrent invasive infections - I'm kind of inclined to believe that most C2-deficient patients don't have recurrent infections (that's why we don't see them) and the exceptional patients who do have another contributing problem like (yes, Troy!) MBL deficiency....

Prescott

 Sent from my iPhone

On Apr 14, 2015, at 10:04 AM, Church, Joseph <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:

Hi Andy.  Thank you for the kind words.  I have seen 2 primary C2 deficiencies over the past 39 years.  They presented with recurrent S. pneumoniae sepsis, as has been reported.  JC

From: Saxon, Andy M.D. [mailto:ASaxon at mednet.ucla.edu]
Sent: Tuesday, April 14, 2015 7:58 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] IVIG/ScIg for Complement deficiency?

As Joe said, makes no sense.  There is a growing group of less than critical folks who are using IVIG (and charging for it through their practices) for all sorts of inappropriate diagnoses and making “appropriate” diagnoses, e.g. CIDP) with little basis so as to give IVIG as a revenue stream.

How many cases of real C2 deficiency have you got floating around up there anyway? And I thought the concern was autoimmunity, not infection in real C2 deficiency as opposed to say C8 deficiency. I’d like to hear from some of you great Peds folks in addition to the outstanding Joe Church..

Andy Saxon, MD
Professor of Medicine
UCLA

PS – Hi Joe!

From: Church, Joseph [mailto:JChurch at chla.usc.edu]
Sent: Tuesday, April 14, 2015 7:51 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] IVIG/ScIg for Complement deficiency?

This makes no sense to me.  The patients’ problem is not the inability to make antibodies, it is the inability to use complement that is activated by the classical pathway (or lectin pathway) regardless of the source of antibodies.

Joe Church
Children’s Hospital Los Angeles

From: Jason Raasch [mailto:jraasch at midwestimmunology.com]
Sent: Monday, April 13, 2015 1:31 PM
To: CIS-PIDD
Subject: [cis-pidd] IVIG/ScIg for Complement deficiency?

Hello everyone.

I have heard of some practitioners in our region using IVIG as sole management for isolated C2 deficiency in infants/children (i.e. instead of prophylactic antibiotics, immunizations for encapsulated organisms, etc.).  The feeling is long-term use of prophylactic antibiotics in these cases is irresponsible.

I will be honest and admit I have not heard of this before or found much in the literature.  What am I missing here?

I would appreciate any comments.

Regards,

Jason

Jason Raasch, MD

Midwest Immunology Clinic
15700 37th Ave N, Ste 110
Plymouth, MN 55446

TEL: (763) 577-0008
FAX: (763) 577-0192

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