Fatal autoimmune pneumonitis requiring bilobectomy and omental flap repair in a patient with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)
| Autor: | Michail S. Lionakis, Jeffrey G. Walls, Huy M. Do, Stephanie A. Kubala, Elise M. N. Ferré, David S. Schrump, Kenneth N. Olivier, Les R. Folio |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Pulmonary and Respiratory Medicine
medicine.medical_specialty animal structures Case Report medicine.disease_cause Immune tolerance Autoimmunity Immunomodulation Diseases of the respiratory system AIRE Bronchopleural fistula medicine APS-1 Pneumonitis Bronchiectasis RC705-779 business.industry Dystrophy Autoimmune polyendocrinopathy Autoimmune regulator medicine.disease Dermatology Omental flap repair Autoimmune pneumonitis Complication business APECED |
| Zdroj: | Respiratory Medicine Case Reports, Vol 33, Iss, Pp 101476-(2021) Respiratory Medicine Case Reports |
| ISSN: | 2213-0071 |
| 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: | OpenAIRE |
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