Fatal autoimmune pneumonitis requiring bilobectomy and omental flap repair in a patient with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)

Fatal autoimmune pneumonitis requiring bilobectomy and omental flap repair in a patient with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)

We present a severe case of progressive autoimmune pneumonitis requiring surgical intervention in a patient with the monogenic syndrome, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). APECED is caused by loss-of-function mutations in the autoimmune regulator (AIRE) gene, wh...

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Bibliographic Details
Main Authors: Stephanie A. Kubala, Huy M. Do, Elise M.N. Ferré, David S. Schrump, Kenneth N. Olivier, Jeffrey G. Walls, Michail S. Lionakis, Les R. Folio
Format: Article
Language:English
Published: Elsevier 2021-01-01
Series:Respiratory Medicine Case Reports
Subjects:
APECED
APS-1
AIRE
Autoimmune pneumonitis
Bronchiectasis
Bronchopleural fistula
Online Access:http://www.sciencedirect.com/science/article/pii/S2213007121001386

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