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Increased intracellular oxygen radical production in neutrophils during febrile episodes of PFAPA syndrome.

Journal article
Authors Martina Sundqvist
Per Wekell
Veronica Osla
Johan Bylund
Karin Christenson
Karin Sävman
Dirk Foell
David A Cabral
Anders Fasth
Stefan Berg
Kelly Brown
Anna Karlsson
Published in Arthritis and rheumatism
Volume 65
Issue 11
Pages 2971–2983
ISSN 1529-0131
Publication year 2013
Published at Institute of Neuroscience and Physiology, Department of Physiology
Institute of Medicine, Department of Rheumatology and Inflammation Research
Institute of Clinical Sciences, Department of Pediatrics
Pages 2971–2983
Language en
Subject categories Immunobiology, Rheumatology and Autoimmunity, Pediatrics


Objective: Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome is an autoinflammatory disease of unknown etiology that primarily affects preschool children. PFAPA is characterized by recurrent attacks of fever and inflammatory symptoms consistent with the disease acronym. Since autoinflammatory diseases by definition are mediated by cells of the innate immune system, we aimed at evaluating functional features of neutrophils, the most abundant innate immune cell in circulation, in PFAPA syndrome. Methods: Blood neutrophils, obtained from PFAPA patients during both febrile and asymptomatic afebrile phases of disease, as well as from healthy children (afebrile controls) and children with fever and abdominal pain (febrile controls) were analysed for three key neutrophil characteristics: (i) apoptosis (measured by Annexin V/7AAD staining), (ii) production of reactive oxygen species (ROS; by luminol/isoluminol-amplified chemiluminescence), and (iii) priming status (as responsiveness to galectin-3 and upregulation of CD11b). Results: Compared to neutrophils from both PFAPA patients in an afebrile interval and from febrile controls, neutrophils obtained during a PFAPA flare produced elevated levels of intracellular NADPH-oxidase-derived ROS, had significantly diminished rates of spontaneous apoptosis, and displayed signatures of priming. In contrast, neutrophils from afebrile PFAPA patients had a significantly elevated rate of spontaneous apoptosis compared to neutrophils from afebrile controls. Conclusions: We demonstrate that three key aspects of neutrophil innate immune function, namely apoptosis, priming, and generation of an intracellular oxidative burst are altered, most prominently during febrile attacks in PFAPA syndrome. © 2013 American College of Rheumatology.

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