Raised Serum Markers of T Cell Activation and Exhaustion in Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency.

Autor: Fraz MSA; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway. mai.sasaki.aanensen@gmail.com.; Centre for Rare Diseases, Oslo University Hospital, Oslo, Norway. mai.sasaki.aanensen@gmail.com., Michelsen AE; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway., Moe N; Department of Radiology and Nuclear Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway., Aaløkken TM; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.; Department of Radiology and Nuclear Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway., Macpherson ME; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Department of Infectious Diseases, Oslo University Hospital Ullevål, Oslo, Norway., Nordøy I; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway., Aukrust P; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.; Faculty of Health Sciences, K.G. Jebsen TREC, University of Tromsø, Tromsø, Norway., Taraldsrud E; Department of Immunology, Oslo University Hospital, Oslo, Norway., Holm AM; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.; Department of Pulmonary Medicine, Oslo University Hospital, Oslo, Norway., Ueland T; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway.; Faculty of Health Sciences, K.G. Jebsen TREC, University of Tromsø, Tromsø, Norway., Jørgensen SF; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway., Fevang B; Section of Clinical Immunology and Infectious Diseases, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Centre for Rare Diseases, Oslo University Hospital, Oslo, Norway.; Research Institute of Internal Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Jazyk: angličtina
Zdroj: Journal of clinical immunology [J Clin Immunol] 2022 Oct; Vol. 42 (7), pp. 1553-1563. Date of Electronic Publication: 2022 Jul 05.
DOI: 10.1007/s10875-022-01318-1
Abstrakt: 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.
(© 2022. The Author(s).)
Databáze: MEDLINE
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