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



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.



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.



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.
Department of Pediatrics, NU-Hospital Group, Uddevalla Department of Pediatrics, Institute of Clinical Sciences.
Per
P
Wekell
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Halla
H
Björnsdottir
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Lena
L
Björkman
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Martina
M
Sundqvist
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Karin
K
Christenson
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Veronica
V
Osla
Department of Pediatrics, Institute of Clinical Sciences The Queen Silvia Children's Hospital.
Stefan
S
Berg
Department of Pediatrics, Institute of Clinical Sciences The Queen Silvia Children's Hospital.
Anders
A
Fasth
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg.
Amanda
A
Welin
Department of Oral Microbiology and Immunology, Institute of Odontology, University of Gothenburg, Gothenburg, Sweden.
Johan
J
Bylund
Department of Rheumatology and Inflammation Research, Institute of Medicine, University of Gothenburg anna.karlsson@rheuma.gu.se.
Anna
A
Karlsson
D020083:Acquired Hyperostosis Syndrome / Q000201:enzymology*; D000209:Acute-Phase Proteins / Q000378:metabolism; D000293:Adolescent; D000368:Aged; D017209:Apoptosis / Q000502:physiology; D015415:Biomarkers / Q000378:metabolism; D016022:Case-Control Studies; D016207:Cytokines / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019255:NADPH Oxidases / Q000096:biosynthesis / Q000378:metabolism*; D009504:Neutrophils / Q000201:enzymology*; D017382:Reactive Oxygen Species / Q000378:metabolism*; D012008:Recurrence; D015854:Up-Regulation / Q000502:physiology
D000209:Acute-Phase Proteins; D015415:Biomarkers; D016207:Cytokines; D017382:Reactive Oxygen Species; D019255:NADPH Oxidases
NADPH oxidase; SAPHO syndrome; autoinflammation; granulocytes; inflammation; reactive oxygen species