7-days of FREE Audio papers, translation & more with Prime
7-days of FREE Prime access
7-days of FREE Audio papers, translation & more with Prime
7-days of FREE Prime access
https://doi.org/10.37897/rjr.2022.1.8
Copy DOIJournal: Romanian Journal of Rheumatology | Publication Date: Mar 31, 2022 |
License type: cc-by |
Haemophagocytic lymphohistiocytosis (HLH) is an underrecognized hyperinflammatory condition with a high mortality, characterized by inappropriate survival of histiocytes and cytotoxic T cells (CTLs), leading to a cytokine storm, haemophagocytosis and multi-organ damage (1). The currently accepted terminology includes two forms of HLH: the primary or familial HLH (fHLH) and the secondary HLH (sHLH) (2). The sHLH associated with rheumatic conditions is known as macrophage activation syndrome (MAS). MAS can develop at any time during the evolution of the rheumatic diseases: at the beginning, during flare of the disease or during intercurrent infections – mostly with viral trigger. We present a case of MAS in an patient with adult-onset Still Disease. It is of great importance to be recognized in early stages, because if promptly treated it might respond well to combination therapy of glucocorticoids and IL1- blockers.
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.