1.
Allergy
; 78(5): 1369-1371, 2023 05.
Artigo
em Inglês
| MEDLINE
| ID: mdl-36510404
2.
J Clin Immunol
; 36(7): 733-8, 2016 10.
Artigo
em Inglês
| MEDLINE
| ID: mdl-27492372
RESUMO
The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.