High levels of interferon-gamma (IFNγ) in macrophage activation syndrome (MAS) and CXCL9 levels as a biomarker for IFNγ production in MAS
نویسندگان
چکیده
Objectives Given the similarities between primary and secondary HLH (sec-HLH), including MAS, we measured levels of IFNg, IFNg-related chemokines (CXCL9, CXCL10, CXLC11), and IL-6 in patients with sec-HLH, and in patients with systemic Juvenile Idiopathic Arthritis (sJIA) with or without MAS at sampling and evaluated their relation to disease activity. In addition, we evaluated the correlation between serum levels of IFNg and of the three IFNg related chemokines with themselves and with laboratory parameters of disease activity in patients with active MAS.
منابع مشابه
Macrophage Activation Syndrome as the First Presentation of Juvenile Idiopathic Arthritis
Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent. A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspira...
متن کاملAnti interferon-gamma (IFNγ) monoclonal antibody treatment in a patient carrying an NLRC4 mutation and severe hemophagocytic lymphohistiocytosis
Background A growing body of evidence, in animals and humans, suggest that IFNg plays a pathogenic role in primary HLH (pHLH) and in secondary HLH, including macrophage activation syndrome. A pilot study in pHLH with NI-0501, an anti-IFNg monoclonal antibody, is ongoing. Mutations in NLRC4 have been recently reported to cause recurrent MAS and an increased production of IL-18, a cytokine known ...
متن کاملInterferon-gamma (IFNy) in macrophage activation syndrome (MAS) associated with systemic juvenile idiopathic arthritis (sJIA). High levels in patients and a role in a murine mas model
Interferon-gamma (IFNg) in macrophage activation syndrome (MAS) associated with systemic juvenile idiopathic arthritis (sJIA). High levels in patients and a role in a murine mas model Claudia Bracaglia, Ivan Caiello, Kathy De Graaf, Giovanni D’Ario, Florence Guilhot, Walter Ferlin, Lidia Melli, Giusi Prencipe, Sergio Davì, Grant Schulert, Angelo Ravelli, Alexei Grom, Cristina De Min, Fabrizio D...
متن کاملInterferon gamma (IFNg) drives disease in the TLR9-mediated cytokine storm syndrome in mice
The Cytokine Storm Syndrome (CSS) is characterized by an overwhelming activation of immune cells observed in life-threatening disorders such as familial hemophagocytic lymphohistiocytosis (fHLH) and secondary (s) HLH/ macrophage activation syndrome (MAS) as well as during serious infection. However, it is not known if the CSS can be attributed to a single cytokine. Increased blood levels of int...
متن کاملMacrophage Activation Syndrome as the First Impression of Kawasaki Disease; A Case Report
Introduction Macrophage activation syndrome (MAS) is a rare and life-threatening complication of Kawasaki Disease (KD) that is usually diagnosed at the same time or after KD. We report a case of MAS as the initial manifestation of KD. Case Report A previously healthy 3-year old girl was admitted to the pediatric infectious di...
متن کامل