[CIS PIDD] APECED & lung transplantation

[Desa Lilic]

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<mailto:desa.lilic at ncl.ac.uk>

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