Expansion of inflammatory innate lymphoid cells in XXXX with common variable immune deficiency.

@entity1 @entity32 @entity67570 @entity13236 @entity6078 @entity14283 @entity4155 @entity379 @entity1968
BACKGROUND: @entity6078 ( @entity6078 ) is an antibody deficiency treated with immunoglobulin; however, @entity1 can have noninfectious inflammatory conditions that lead to heightened morbidity and mortality. OBJECTIVES: Modular analyses of RNA transcripts in whole blood previously identified an upregulation of many interferon-responsive genes. In this study we sought the cell populations leading to this signature. METHODS: Lymphoid cells were measured in peripheral blood of 55 @entity1 with @entity6078 (31 with and 24 without inflammatory/autoimmune complications) by using mass cytometry and flow cytometry. Surface markers, cytokines, and transcriptional characteristics of sorted innate lymphoid cells (ILCs) were defined by using quantitative PCR. Gastrointestinal and lung biopsy specimens of subjects with @entity32 were stained to seek ILCs in tissues. RESULTS: The linage-negative, CD127(+), CD161(+) lymphoid population containing T-box transcription factor, @entity1968 -related orphan receptor (ROR) yt, @entity379 , @entity4155 , and @entity13236 , all hallmarks of type 3 innate lymphoid cells, were expanded in the blood of @entity1 with @entity6078 with inflammatory conditions (mean, 3.7% of PBMCs). ILCs contained detectable amounts of the transcription factors inhibitor of DNA binding 2, T-box transcription factor, and RORyt and increased mRNA transcripts for @entity14283 ( @entity14283 ) and @entity67570 , demonstrating inflammatory potential. In gastrointestinal and lung biopsy tissues of @entity1 with @entity6078 , numerous @entity379 (+)RORyt(+)CD3(-) cells were identified, suggesting a role in these mucosal inflammatory states. CONCLUSIONS: An expansion of this highly inflammatory ILC population is a characteristic of @entity1 with @entity6078 with @entity32 ; ILCs and the interferon signature are markers for the uncontrolled inflammatory state in these @entity1 .
