[CIS PIDD] APECED & lung transplantation

[Elie Haddad]

Dear Desa,
I think lung transplantation will be tough in your case, and the logical idea to do lung and HSCT with the same donor does not seem very feasible.
However, about Rituximab, I would advise you to try it in your patient. We've had a patient with APECED and it worked during many years for her multiple autoimmunity and the patient does not want to stop it since she says it's the best treatment she ever had and all her endocrinological and metabolic disturbances are stabilized by Rituximab. Will it work also for interstitial lung disease ? I don't know, but the idea to do like in GLILD associated with CVID with Rituximab and Imuran seems a logical option. I would say you have not much lo loose.
Interestingly, (and it was in 2006) the patient told me that after Rituximab, her severe candidiasis vanished. To tell you the truth, I thought it was "psychological". Before giving Rituximab, I was concerned by this candidiasis, but we felt that killing B cells should not be too dangerous for her candidiasis. However, we did not think that it would cure her candidiasis. Now that we know that there are anti-IL-17 autoantibodies in this disease, it sounds logical. Therefore, Rituximab maybe will help for lung autoimmunity and maybe will help for clearing the candidiasis before the transplantation if you decide to do it.
I hope it will help
All the best
Elie


Elie Haddad, MD, PhD,
Professor of Pediatrics, University of Montreal,
Head, Pediatric Immunology and Rheumatology Division,
CHU Sainte-Justine, 3175 Cote Sainte-Catherine
Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca





Le 2012-07-26 à 04:47, Desa Lilic a écrit :


> Thnx for reply. I am aware of a clinical trial in Great Ormond Street (GOS - London) of thymic transplants in Di George but not in APECEDs

>  

> best

> desa

> From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Church, Joseph [JChurch at chla.usc.edu]

> Sent: 25 July 2012 18:18

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS PIDD] APECED & lung transplantation

> 

> Theoretically, a thymus transplant should help and may be less risky than BMT.  Unfortunately, I don't know any centers doing thymus transplant in APECED. 

>  

> Joe Church

> Children's Hospital Los Angeles

>  

> From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Desa Lilic

> Sent: Wednesday, July 25, 2012 3:30 AM

> To: pagid at list.clinimmsoc.org

> Subject: [CIS PIDD] APECED & lung transplantation

>  

> Dear all,

>  

> We have a young lady (26 yr) who has antibody deficiency & APECED - MC,

> HP, Addison, Autoimmune hepatitis, Lymphocytic Interstitial pneumonitis

> and autoimmune ovarian failure (see attached summary for more details).

>  

> She has bad bronchiectasis ( being managed by our CF colleagues) with

> deteriorating lung function.  Given the rapidity of deterioration in her

> lung function, the lung physicians  are considering a referral

> for assessment for a lung transplant.

>  

> 1.  Are you aware any lung transplantations in the AIRE Mutation /

> APECED cohort ?  (no published reports; I have liaised Jaakko

> Perheentupa - none in the Finnish cohort; )

>  1.  Risk of invasive candidiasis with significant immunesuppresion (

> >aware of the report on renal transplant - there was no invasive

> >candidiasis)

>  1.  Prognosis and life expectancy of a typical APECED patient with HP,

> Adr failure, autoimmune hepatitis. To make an reasoned case for lung

> >transplant

> 1.  Thoughts on simultaneous Thymic transplant  ?

> 1.  Does the diagnosis of APECED in any way influence ones decision

> making process for eligibility of a lung transplant and risk benefit

> analysis ?

> 1.  She is on Azathioprine for autoimmune hepatitis.  One of the

> discussion points in our MDT  was whether she would benefit form

> rituximab - to rx a potential ongoing interstitial inflammation in her

> lung.

>  

> Would be grateful for your thoughts / comments.

> Desa

>  

> Desa Lilic, MD, MSc, PhD, FRCPath

> Consultant & Clinical Senior Lecturer in Immunology

> Institute for Cellular Medicine

> 4th Fl Cookson Bldg

> Faculty of Medical Sciences

> University of Newcastle

> Newcastle upon Tyne, UK, NE2 4HH

> 

> tel: ++ 44 (0)191 222 7244

> fax: ++ 44 (0)191 222 5455

> email: desa.lilic at ncl.ac.uk

>  

> 

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