Sofie Struyf
University of Leuven, Belgium
Title: The chronic inflammatory milieu in the lungs of patients with Primary Ciliary Dyskinesia (PCD) causes alterations in innate immune responses
Biography
Biography: Sofie Struyf
Abstract
Patients suffering from Primary Ciliary Dyskinesia (PCD), an autosomal inherited disorder caused by defective motile cilia, often have to cope with recurrent infections of the upper and lower airways. We investigated the innate immune system in PCD pathology. More precisely, we verified whether the chronic inflammatory environment in PCD patients affects the activity of blood leukocytes in their defense against pathogens. We detected abnormalities in the responses of innate immune cells (neutrophils and monocytes purified from peripheral blood) from PCD patients. Both neutrophilic granulocytes and monocytes showed an altered expression pattern of chemoattractant receptors. In addition, neutrophils and monocytes from PCD patients reacted differently than those from healthy controls in terms of upregulation of adhesion molecules in response to chemotactic stimuli. But, only PCD neutrophilic granulocytes were affected in their migratory capacity. Finally, PCD patients have a higher proportion of inflammatory monocytes and those cells release higher amounts of inflammatory cytokines after stimulation with TLR ligands. We can thus conclude that innate immune cells from PCD patients act differently from those of healthy individuals and that chronic airway inflammation alters innate immune responses.