Dysregulated neutrophil responses and neutrophil extracellular trap formation and degradation in PAPA syndrome.

ABSTRACT:

Objectives

Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is characterised by flares of sterile arthritis with neutrophil infiltrate and the overproduction of interleukin (IL)-1β. The purpose of this study was to elucidate the potential role of neutrophil subsets and neutrophil extracellular traps (NET) in the pathogenesis of PAPA.

Methods

Neutrophils and low-density granulocytes (LDG) were quantified by flow cytometry. Circulating NETs were measured by ELISA and PAPA serum was tested for the ability to degrade NETs. The capacity of NETs from PAPA neutrophils to activate macrophages was assessed. Skin biopsies were analysed for NETs and neutrophil gene signatures.

Results

Circulating LDGs are elevated in PAPA subjects. PAPA neutrophils and LDGs disp

SUBMITTER: Mistry P 

PROVIDER: S-EPMC6728908 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature