Anti-topoisomerase, but not anti-centromere B cell responses in systemic sclerosis display active, Ig-secreting cells associated with lung fibrosis.
Corrie M WortelSophie I E LiemNina Marijn van LeeuwenMaaike BoonstraCynthia M FehresLauran StögerThomas Wj HuizingaRené E M ToesJeska De Vries-BouwstraHans Ulrich SchererPublished in: RMD open (2023)
In contrast to ACA-positive patients, ATA-positive patients show signs of recent activation of the B cell response that hallmarks this disease. The degree of activation correlates with the presence and severity of ILD, the most deleterious disease manifestation. This could explain differential responsiveness to B cell depleting therapy. The abundant and spontaneous secretion of ATA-IgG and ATA-IgA may point toward a continuously activating trigger.
Keyphrases
- systemic sclerosis
- end stage renal disease
- ejection fraction
- chronic kidney disease
- interstitial lung disease
- newly diagnosed
- induced apoptosis
- prognostic factors
- healthcare
- signaling pathway
- mesenchymal stem cells
- magnetic resonance
- oxidative stress
- endoplasmic reticulum stress
- cell cycle arrest
- contrast enhanced
- pi k akt