Objectives Interferon-gamma (IFN gamma) is the pivotal mediator in murine models of primary haemophagocytic lymphohistiocytosis (pHLH). Given the similarities between primary and secondary HLH (sec-HLH), including macrophage activation syndrome (MAS), we investigate the involvement of the IFN gamma pathway in MAS by evaluating levels of IFN...
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2017 (v1)PublicationUploaded on: March 27, 2023
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2018 (v1)Publication
Introduction Autoinflammatory diseases can cause irreversible tissue damage due to systemic inflammation. Recently, the Autoinflammatory Disease Damage Index (ADDI) was developed. The ADDI is the first instrument to quantify damage in familial Mediterranean fever, cryopyrin-associated periodic syndromes, mevalonate kinase deficiency and tumour...
Uploaded on: April 14, 2023