Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease

Assari, Raheleh; Ziaee, Vahid; Mirmohammadsadeghi, Arash; Moradinejad, Mohammad-Hassan

doi:10.1155/2015/424381

Cited by 18 publications

(19 citation statements)

References 28 publications

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“…Nine papers were excluded after full text screening. Two papers on biomarkers were excluded because data of sJIA patients with MAS were inseparably mixed with MAS in the context of other underlying diseases [ 14 , 15 ]. 7 papers assessing genetic associations of MAS in sJIA were excluded because these papers addressed possible genetic associations as possible underlying causes rather than as possible biomarkers for diagnosis, disease activity or treatment response.…”

Section: Resultsmentioning

confidence: 99%

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

et al. 2015

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BackgroundMacrophage activation syndrome (MAS) is a severe and potentially lethal complication of several inflammatory diseases but seems particularly linked to systemic juvenile idiopathic arthritis (sJIA). Standardized diagnostic and treatment guidelines for MAS in sJIA are currently lacking. The aim of this systematic literature review was to evaluate currently available literature on diagnostic criteria for MAS in sJIA and provide an overview of possible biomarkers for diagnosis, disease activity and treatment response and recent advances in treatment.MethodsA 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.Results27 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 MAS in sJIA. 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 MAS from active sJIA and/or infection with superior performance.Other promising diagnostic biomarkers potentially distinguish MAS complicating sJIA from primary and virus-associated hemophagocytic lymphohistiocytosis.The highest level of evidence for treatment comes from case-series. High dose corticosteroids with or without cyclosporine A were frequently reported as first-line therapy. From the newer treatment modalities, promising responses have been reported with anakinra.ConclusionMAS in sJIA seems to be diagnosed best by the recently proposed PRINTO criteria, although prospective validation is needed. Novel promising biomarkers for sJIA related MAS are in need of prospective validation as well, and are not widely available yet. Currently, treatment of MAS in sJIA relies more on experience than evidence based medicine. Taking into account the severity of MAS and the scarcity of evidence, early expert consultation is recommended as soon as MAS is suspected.

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Section: Resultsmentioning

confidence: 99%

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

et al. 2015

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“…Most of the studies supported subtle laboratory alterations and monitoring of the laboratory changes, along with an early recognition of the clinical presentations for MAS onset; these were crucial for an early diagnosis of MAS 5,33,35,36. The laboratory parameters, including platelet count, as well as the aspartate and alanine aminotransferases, together with the ferritin, lactate dehydrogenase, triglyceride, and d -dimer levels, showed >50% changes between a pre-MAS visit and MAS onset 10.…”

Section: Clinical and Laboratory Features: How To Recognize Mas Earlymentioning

confidence: 99%

Macrophage activation syndrome: early diagnosis is key

Lerkvaleekul¹,

Vilaiyuk²

2018

OARRR

114

103

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Macrophage activation syndrome (MAS) is a life-threatening condition, and it is a subset of hemophagocytic lymphohistiocytosis (HLH). The clinical features include a persistent high-grade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsis-like condition. From the clinical features, it is usually difficult to differentiate between a true sepsis, disease flare-ups, or MAS. Although the laboratory abnormalities are similar to those of a disseminated intravascular coagulation, which shows pancytopenia, coagulopathy, hypofibrinogenemia, and an elevated d-dimer test, it can also be a late stage of MAS. Currently, MAS is still underrecognized and usually results in delayed in diagnosis, which leads to high morbidity and mortality. This literature review was conducted in the context of the clinical manifestations and the laboratory abnormalities in MAS, which might provide some clues for an early diagnosis. The best ways for an early recognition and a satisfactory diagnosis were based on the relative changes in the overall parameters from the baseline, together with a thorough and continuous physical examination for these kinds of patients. At present, diagnostic criteria have been proposed for HLH, MAS-associated systemic juvenile idiopathic arthritis, and an MAS-associated systemic lupus erythematosus. Therefore, selecting the proper diagnostic criteria for use is essential because not all of the criteria are suitable for every autoimmune disease.

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“…For example, neutrophils and platelets may be within the laboratory normal range but it is the drop from previously elevated levels associated with acute infection or active autoimmune disease that may herald development of HLH 33. The paradoxically falling ESR despite increasing systemic inflammation is thought to be secondary to decreasing fibrinogen resulting from fibrinogen consumption and liver dysfunction 4.…”

Section: Clinical Features and Diagnosismentioning

confidence: 99%

Diagnosing haemophagocytic syndrome

2016

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Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment. Distinguishing HLH from systemic sepsis can present a major challenge. Criteria for diagnosis and classification of HLH and MAS are available and a serum ferritin >10 000 µg/L is strongly supportive of HLH. Without early recognition and appropriate treatment, HLH is almost universally fatal. However, with prompt referral and advancements in treatment over the past two decades, outcomes have greatly improved.

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Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease

Cited by 18 publications

References 28 publications

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Macrophage activation syndrome: early diagnosis is key

Diagnosing haemophagocytic syndrome

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