[CIS-PAGID] Patient with Selective Antibody deficiency andautoimmune disorder

[Paris, Kenneth]

I would agree that immunoglobulin will cover the antibody defect, and that if her diagnosis is truly limited to SAD, then there isn't too much risk over what we would anticipate in the usual patient.  But, I may keep a closer eye on her.  I wouldn't follow efficacy of IVIG using "trough" levels however, since pts with SAD have normal IgG at onset of therapy.   
 
I'm a little concerned about the "undefined inflammatory process" diagnosis, however.   ESR is elevated while on immunoglobulin replacement, and therefore CRP is a better test, but isn't specific at all.  I don't think an elevation in either is justification for use of immunomodulators, especially if there isn't a well defined diagnosis or a constellation of symptoms that points to to a specific disease.  We've used immunomodulators (very few) in some ab deficient patients, usually for inflammatory bowel disease or similar organ specific inflammation.  Tese patients have been mostly classic CVID patients, not SAD patients.  
 
Ken
 
Kenneth Paris MD, MPH
Assistant Professor of Pediatrics
A/I Fellowship Training Program Director
Division of Allergy and Immunology
LSU Health Sciences Center
Children's Hospital of New Orleans
 
Mail: 
200 Henry Clay Avenue
Children's Hospital 
Research Institute for Children 4th Floor
New Orleans, LA 70118
Phone:  504-896-9589
Fax:  504-896-9311
Email:  kparis at lsuhsc.edu <mailto:kparis at lsuhsc.edu> 
 
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________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Church, Joseph
Sent: Wed 3/7/2012 5:00 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] Patient with Selective Antibody deficiency andautoimmune disorder



Colleagues:

 

I have followed a 40yo woman with selective antibody deficiency for several months.  Despite excellent IgG levels on Hizentra (>2000mg/dL) she continues to have recurrent, but non-serious URIs.

 

Her rheumatologist sees her for an undefined inflammatory process, perhaps RA or evolving SLE.  He wishes to treat the patient with a TNF blocker or Imuran, but is concerned about the potential for increased risk for serious infections because of the patient's antibody deficiency.

 

My sense is that the patient's antibody deficiency is well covered by the Hizentra and that the risk for infection is perhaps marginally increased over the usual risks with these agents.

 

I would very much appreciate your thoughts on this issue.

 

Joe Church

Children's Hospital Los Angeles

 

 


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