1.
Allergy
; 78(5): 1369-1371, 2023 05.
Article
in English
| MEDLINE
| ID: mdl-36510404
2.
J Clin Immunol
; 36(7): 733-8, 2016 10.
Article
in English
| MEDLINE
| ID: mdl-27492372
ABSTRACT
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.