[CIS PIDD] APECED & lung transplantation
Desa Lilic
desa.lilic at newcastle.ac.uk
Wed Jul 25 06:13:12 EDT 2012
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 ( she is positive for an antibody against LPLUNC1 protein).
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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