| Autor: |
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 |
| Jazyk: |
angličtina |
| Rok vydání: |
2021 |
| Předmět: |
|
| Zdroj: |
Respiratory Medicine Case Reports, Vol 33, Iss , Pp 101476- (2021) |
| Druh dokumentu: |
article |
| ISSN: |
2213-0071 |
| DOI: |
10.1016/j.rmcr.2021.101476 |
| Popis: |
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, which lead to impaired central immune tolerance and autoimmune organ destruction including pneumonitis, an underrecognized, life-threatening complication. When clinicians evaluate patients with pneumonitis, recurrent mucosal candidiasis, and autoimmunity, APECED should be considered in the differential. Additionally, in patients with established APECED, a chest computed tomography is preferred to identify pneumonitis early on and to promptly initiate lymphocyte-directed immunomodulatory treatment, which can prevent irreversible lung destruction. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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