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Article

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

Journal of clinical immunology - 1 Oct 2016

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

Abstract excerpt

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...

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Source

Identifiers

PMID: 27492372
DOI: 10.1007/s10875-016-0320-3

Genes

XIAP

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.PMID 27492372Read ↗

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Phenotype, genotype, treatment, and survival outcomes in patients with X-linked inhibitor of apoptosis deficiency.Semantic proximity Read ↗

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease.Semantic proximity Read ↗

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Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAP.Semantic proximity Read ↗

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