[CIS PIDD] [cis-pidd] lung transplant in CVID patients with GLID?

[Elena Hsieh]

Thanks Dr. Routes.  Our patient received four courses of rituximab, and about 6-8 months of imuran; she was getting better then recently deteriorated and developed pulmonary hypertension.  I have indeed found reports of lung transplant for bronchiectasis in CVID, the issue is whether that experience could be extrapolated to GLILD.  

Thanks for your thoughts.

Elena

> On Jul 16, 2015, at 07:52, Routes, John <jroutes at mcw.edu> wrote:
> 
> HI Elena
> I am not aware of any studies on this, although my guess is there are
> isolated case reports‹there are reports on lung transplantion in CVID and
> bronchiectasis with or without hypogammaglobulinemia‹
> you didnt mention how many courses of RTX the patient got and how long Rx
> with immuran‹‹we have retreated some or our patients
> also, might want to consider pulse methylprednisolone and see if she has
> any reversible component
> good luck
> 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
> 
> 
> 
> 
> On 7/16/15, 12:02 AM, "Elena Hsieh" <whsieh at stanford.edu> wrote:
> 
>> Hi there,
>> 
>> We have a very sick 48yo woman with CVID and granulomatous-lymphocytic
>> interstitial lung disease (GLILD), who has now developed pulmonary
>> hypertension, s/p rituximab and imuran, currently not doing well.  Is
>> there a precedent for lung transplant in CVID patients with GLILD who
>> fail improvement with immunosuppressive regimen? If so, could you point
>> me to a good reference?
>> 
>> Thanks.
>> Elena
>> 
>> Instructor
>> Allergy and Immunology
>> Stanford University
>> 
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