Raised Serum Markers of T Cell Activation and Exhaustion in Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency.
J Clin Immunol
; 42(7): 1553-1563, 2022 10.
Article
em En
| MEDLINE
| ID: mdl-35789314
ABSTRACT
PURPOSE:
About 20-30% of patients with common variable immunodeficiency (CVID) develop granulomatous-lymphocytic interstitial lung disease (GLILD) as one of several non-infectious complications to their immunodeficiency. The purpose of this study was to identify biomarkers that could distinguish GLILD from other non-infectious complications in CVID.METHODS:
We analyzed serum biomarkers related to inflammation, pulmonary epithelium injury, fibrogenesis, and extracellular matrix (ECM) remodeling, and compared three subgroups of CVID GLILD patients (n = 16), patients with other non-infectious complications (n = 37), and patients with infections only (n = 20).RESULTS:
We found that GLILD patients had higher levels of sCD25, sTIM-3, IFN-γ, and TNF, reflecting T cell activation and exhaustion, compared to both CVID patients with other inflammatory complications and CVID with infections only. GLILD patients also had higher levels of SP-D and CC16, proteins related to pulmonary epithelium injury, as well as the ECM remodeling marker MMP-7, than patients with other non-infectious complications.CONCLUSION:
GLILD patients have elevated serum markers of T cell activation and exhaustion, pulmonary epithelium injury, and ECM remodeling, pointing to potentially important pathways in GLILD pathogenesis, novel targets for therapy, and promising biomarkers for clinical evaluation of these patients.Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Imunodeficiência de Variável Comum
/
Doenças Pulmonares Intersticiais
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
Limite:
Humans
Idioma:
En
Revista:
J Clin Immunol
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Noruega