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05.08.2016 | Original Article

X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis

verfasst von: Cathal L. Steele, Matthew Doré, Sandra Ammann, Maurice Loughrey, Angeles Montero, Siobhan O. Burns, Emma C. Morris, Bobby Gaspar, Kimberly Gilmour, Shahnaz Bibi, Hiba Shendi, Lisa Devlin, Carsten Speckmann, David M. Edgar

Erschienen in: Journal of Clinical Immunology | Ausgabe 7/2016

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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.

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Titel: X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis
verfasst von: Cathal L. Steele
Matthew Doré
Sandra Ammann
Maurice Loughrey
Angeles Montero
Siobhan O. Burns
Emma C. Morris
Bobby Gaspar
Kimberly Gilmour
Shahnaz Bibi
Hiba Shendi
Lisa Devlin
Carsten Speckmann
David M. Edgar
Publikationsdatum: 05.08.2016
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 7/2016
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-016-0320-3

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X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis

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