Objective: To investigate the characteristics of circulating natural killer (NK) cells and their interferon (IFN)-γ-producing ability in adult-onset Still disease (AOSD).
Methods: Peripheral blood mononuclear cells were obtained from 22 patients in the acute phase of AOSD (acute AOSD); 7 of the 22 patients after treatment (remission AOSD), and 11 healthy controls (HC). NK cells and their IFN-γ expression levels were analyzed by flow cytometry. Additionally, the cytokine receptors of interleukin (IL)-12, IL-15, and IL-18 on NK cells were also evaluated.
Results: The frequency of NK cells was significantly lower in acute AOSD than in HC. NK cell counts significantly increased in remission AOSD. Expression of IL-12 and IL-15 receptors on NK cells was significantly increased in acute AOSD, whereas that of IL-18 receptor indicated no significant difference among 3 groups. IFN-γ expression in NK cells was significantly higher in acute AOSD than in HC, and significantly decreased in remission AOSD. The absolute number of NK cells and IFN-γ-expressing NK cells revealed an inverse correlation with serum ferritin levels in acute AOSD. In 2 distinct subsets of NK cells, CD56 NK cells significantly exhibited higher IFN-γ expression than CD56 NK cells in acute AOSD.
Conclusion: In acute AOSD, NK cells displayed lower proportion, whereas they had higher ability for IFN-γ production than in HC; moreover, upregulation of IL-12 and IL-15 receptors on NK cells may promote IFN-γ production. In addition, disease activity may be implicated in regulating the number of NK cells and IFN-γ-expressing NK cells in AOSD.
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http://dx.doi.org/10.3899/jrheum.181192 | DOI Listing |
Cureus
November 2024
Diabetes and Endocrinology, United Lincolnshire Hospitals NHS Trust, Boston, GBR.
Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disorder that presents with diverse, overlapping symptoms, complicating the diagnostic process due to its nonspecific clinical features and the absence of a definitive diagnostic test. Diagnosis is often challenging and relies on excluding other conditions while maintaining a high index of suspicion, supported by specific diagnostic criteria such as Yamaguchi or Fautrel. Prompt recognition and a multidisciplinary approach are essential, as AOSD can progress to life-threatening multiorgan dysfunction due to a hyperinflammatory response.
View Article and Find Full Text PDFCureus
November 2024
School of Medicine, Universidad Complutense de Madrid, Madrid, ESP.
Kikuchi-Fujimoto disease (KFD) and adult-onset Still disease (AOSD) are two rare conditions whose association poses a significant diagnostic challenge. KFD is characterized by subacute necrotizing lymphadenitis of unknown etiology, primarily affecting young adults, and often presents with fever and posterior cervical lymphadenopathy. AOSD is a systemic inflammatory disorder of unclear origin, defined by high-spiking fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis.
View Article and Find Full Text PDFFront Immunol
November 2024
Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain.
Objective: Next-generation sequencing (NGS) panels are increasingly used for the diagnosis of monogenic systemic autoinflammatory diseases (SAIDs). However, their role in patients with adult-onset Still's disease (AOSD) remains unknown. This study aims to assess the usefulness of NGS panels in AOSD patients to improve diagnosis and management of the disease.
View Article and Find Full Text PDFCureus
September 2024
Rheumatology, Wyckoff Heights Medical Center, New York City, USA.
Cureus
September 2024
Rheumatology, Wyckoff Heights Medical Center, New York, USA.
Ferritin is commonly used as a marker for iron status, aiding in diagnosing iron deficiency anemia. However, it is also an acute phase reactant often elevated in various inflammatory conditions. Marked hyperferritinemia, defined as ferritin levels above 10,000 μg/L, can indicate severe underlying conditions, including infections, cardiovascular like heart failure, endocrinological, autoimmune, and malignancies.
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