BACKGROUND: @entity15835 ( @entity15835 ) is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to @entity4932 ( @entity4932 ). Standardized diagnostic and treatment guidelines for @entity15835 in @entity4932 are currently lacking. The aim of this systematic literature review was to evaluate currently available literature on diagnostic criteria for @entity15835 in @entity4932 and provide an overview of possible biomarkers for diagnosis, disease activity and treatment response and recent advances in treatment. METHODS: A systematic literature search was performed in MEDLINE, EMBASE and Cochrane. 495 papers were identified. Potentially relevant papers were selected by 3 authors after which full text screening was performed. All selected papers were evaluated by at least two independent experts for validity and level of evidence according to EULAR guidelines. RESULTS: 27 papers were included: 7 on diagnosis, 9 on biomarkers and 11 on treatment. Systematic review of the literature confirmed that there are no validated diagnostic criteria for @entity15835 in @entity4932 . The preliminary Ravelli criteria, with the addition of ferritin, performed well in a large retrospective case-control study. Recently, an international consortium lead by PRINTO proposed a new set of diagnostic criteria able to distinguish @entity15835 from active @entity4932 and/or infection with superior performance. Other promising diagnostic biomarkers potentially distinguish @entity15835 complicating @entity4932 from primary and virus-associated @entity9058 . The highest level of evidence for treatment comes from case-series. High dose @entity288 with or without @entity205 were frequently reported as first-line therapy. From the newer treatment modalities, promising responses have been reported with anakinra. CONCLUSION: @entity15835 in @entity4932 seems to be diagnosed best by the recently proposed PRINTO criteria, although prospective validation is needed. Novel promising biomarkers for @entity4932 related @entity15835 are in need of prospective validation as well, and are not widely available yet. Currently, treatment of @entity15835 in @entity4932 relies more on experience than evidence based medicine. Taking into account the severity of @entity15835 and the scarcity of evidence, early expert consultation is recommended as soon as @entity15835 is suspected.

Evidence-based diagnosis and treatment of macrophage activation syndrome in XXXX .

@entity9058 :: ('MESH:D051359', 'Disease') :: ['hemophagocytic lymphohistiocytosis'] @entity4932 :: ('MESH:D001171', 'Disease') :: ['sJIA', 'systemic juvenile idiopathic arthritis'] @entity205 :: ('MESH:D016572', 'Chemical') :: ['cyclosporine A'] @entity15835 :: ('MESH:D055501', 'Disease') :: ['Macrophage activation syndrome', 'MAS'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity4932 :: (MESH:D001171,Disease) :: ['systemic juvenile idiopathic arthritis']
