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

[Bodo Grimbacher]

Dear Elena,

I am aware of a few cases. I do not think there is a single publication
though.
Outcome is very limited (<5 yrs survival). Our patient survived for one
year, but with a very good QoL.
If I may recommend, please consider to perform a BMT prior to the LungTX.

However, how thoroughly have you excluded e.g. CMV pneumonitis, myclopasma
infection of the lungs?
Have you tried T cell targeted therapies such as sirolimus/cyclosporin/MMF
or CTLA4-Fc, which have been
reported helpful in CVID-associated GLILD?
Also: what was the maximum Korticosteroid does you have used for how long?
If your patient did not respond to 1 mg/kgBW given for 6 months, then I
would follow your statement of non-responding.
Another thought: has one thought of a high-dose IVIG treatment for immune
modulation?

Yours, Bodo
****************************************
Univ.-Prof. Dr. med. B. Grimbacher
Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
www.uniklinik-freiburg.de/cci

and
Consultant Immunologist
Institute of Immunity & Transplantation
Dept of Immunology
Royal Free Hospital
UNIVERSITY COLLEGE LONDON
Pond Street
London NW3 2QG
b.grimbacher at ucl.ac.uk
www.centreforimmunodeficiency.com






Am 16/07/15 16:52 schrieb "Routes, John" unter <jroutes at mcw.edu>:

>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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