[CIS PIDD] [cis-pidd] Lung transplantation in CVID

[CIS-PIDD]

I encountered a similar problem about 25 years ago when I proposed a 35 yo
XLA with severe hepatitis C (at the time nonA nonB) for liver transplant. I
made the argument that IG replacement made him a better candidate because
his infection risk would be decreased compared to other immunosuppressed
transplant recipients who were not receiving IG. He was accepted,
transplanted and lived more than 15 years post-transplant with an excellent
quality of life for most of that time. If the patient does not have
significant comorbidities other than GLILD, I think you could make a
similar argument.
Richard Wasserman
Dallas

On Tue, Mar 1, 2016 at 11:43 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Dear All,
> I have a 42yo male with CIVD that was diagnosed with "sarcoidosis" and
> treated with steroids for many years. When he came to our institution, the
> diagnosis of GLILD was made and we treated him with RTX/MMF (could not
> tolerate azathioprine). Unfortunately, he had moderate to severe pulmonary
> fibrosis with severe restrictive lung disease at the time of presentation
> and is O2 dependent. The immunosuppression has stabilized his lung disease,
> but with a normal decline in lung function with age he will require lung
> transplantation at some point in time. I have referred him for evaluation
> of lung transplantation, but so far two  programs have refused to even
> evaluate him citing CVID as a contraindication. There is a paucity of
> literature on this topic and most lung transplants I could find in the
> literature had other complications at the time of transplantation  (severe
> bronchiectasis, hematological abnormalities etc.,), which my patient does
> not have. He has been on immunosuppressive therapy for a number of years
> w/o serious infections and I believe that the pos-transplant
> immunosuppression (cyclosporin-MMF) would make a return of the GLILD highly
> unlikely. Does anyone have any direct experience in lung transplantation
> for pulmonary fibrosis due to GLILD in CVID.
> thanks
> Jack
>
>
>
> John M. Routes, MD
> Chief, Section of Allergy and Clinical Immunology
> Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
> Department of Pediatrics
> Children's Hospital of Wisconsin
> Medical College of Wisconsin
> 9000 W. Wisconsin Ave.
> Milwaukee, WI  53226-4874
> Phone: Office 414-266-6840
> Fax: 414-266-6437
> Email: jroutes at mcw.edu<mailto:jroutes at mcw.edu>
>
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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
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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