Neutrophils from patients with SAPHO syndrome show no signs of aberrant NADPH oxidase-dependent production of intracellular reactive oxygen species.

Abstract

OBJECTIVE:

We aimed to investigate if aberrant intracellular production of NADPH oxidase-derived reactive oxygen species (ROS) in neutrophils is a disease mechanism in the autoinflammatory disease SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis and osteitis, as has previously been suggested based on a family with SAPHO syndrome-like disease.

METHODS:

Neutrophil function was explored in a cohort of four patients with SAPHO syndrome, two of whom were sampled during both inflammatory and non-inflammatory phase. Intracellular neutrophil ROS production was determined by luminol-amplified chemiluminescence in response to phorbol myristate acetate.

RESULTS:

Cells from all patients produced normal amounts of ROS, both intra- and extracellularly, when compared with internal controls as well as with a large collection of healthy controls assayed in the laboratory over time (showing an extensive inter-personal variability in a normal population). Further, intracellular production of ROS increased during the inflammatory phase. Neutrophil activation markers were comparable between patients and controls.

CONCLUSION:

Dysfunctional generation of intracellular ROS in neutrophils is not a generalizable feature in SAPHO syndrome. Secondly, serum amyloid A appears to be a more sensitive inflammatory marker than CRP during improvement and relapses in SAPHO syndrome.

• Authors: Per Wekell, Halla Björnsdottir, Lena Björkman, Martina Sundqvist, Karin Christenson, Veronica Osla, Stefan Berg, Anders Fasth, Amanda Welin, Johan Bylund, Anna Karlsson
• Journal: Rheumatology (Oxford, England) (Rheumatology (Oxford)) Vol. 55 Issue 8 Pg. 1489-98 (08 2016) ISSN: 1462-0332 [Electronic] England
• PMID: 27121779 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology.

Chemical References

• Acute-Phase Proteins
• Biomarkers
• Cytokines
• Reactive Oxygen Species
• NADPH Oxidases

Topics

• Acquired Hyperostosis Syndrome (enzymology)
• Acute-Phase Proteins (metabolism)
• Adolescent
• Aged
• Apoptosis (physiology)
• Biomarkers (metabolism)
• Case-Control Studies
• Cytokines (metabolism)
• Female
• Humans
• Male
• Middle Aged
• NADPH Oxidases (biosynthesis, metabolism)
• Neutrophils (enzymology)
• Reactive Oxygen Species (metabolism)
• Recurrence
• Up-Regulation (physiology)