Research ArticleAutoimmunity

Lymphocyte-driven regional immunopathology in pneumonitis caused by impaired central immune tolerance

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Science Translational Medicine  05 Jun 2019:
Vol. 11, Issue 495, eaav5597
DOI: 10.1126/scitranslmed.aav5597

A breath of fresh AIRE

Mutations in the AIRE gene lead to APECED, which presents with a variety of autoimmune symptoms. Ferré et al. prospectively studied a large cohort of patients with APECED and discovered that a substantial proportion of them suffered from pneumonitis, which had gone largely undiagnosed. The airways and lung tissue were characterized by infiltration of activated neutrophils and lymphocytes. This infiltration was also observed in Aire−/− mice, and pneumonitis was ablated by T or B cell deficiency. Accordingly, patients treated with drugs to modulate T or B lymphocytes resolved pneumonitis symptoms and improved respiratory function. This study reveals a serious and previously unappreciated manifestation of AIRE deficiency and paves the way for interventions.

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