Identification of the best cutoff points and clinical signs specific for early recognition of macrophage activation syndrome in active systemic juvenile idiopathic arthritis

Kostik, Mikhail M.; Dubko, Margarita; Masalova, Vera; Snegireva, Ludmila; Kornishina, Tatyana; Чикова, И. А.; Likhacheva, Tatyana; Исупова, Е. А.; Glebova, Natalia; Kuchinskaya, Ekaterina; Balbotkina, E. V.; Buchinskaya, Natalia V.; Kalashnikova, Olga V.; Chasnyk, Vyacheslav

doi:10.1016/j.semarthrit.2014.09.004

Cited by 42 publications

(42 citation statements)

References 28 publications

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“…Hepatosplenomegaly and lymphadenopathy are both present in those patients with an active SJIA and MAS, but these abnormalities deteriorate when MAS occurs. However, in a study by Kostik et al,33 these clinical features showed high sensitivity, with poor specificity.…”

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

confidence: 90%

“…Additionally, the relative decreases in platelet count appeared to be the most valuable indication for differentiating between MAS onset and a disease flare-up 7,36. The study by Kostik et al33 showed that a combination of >3 of the following laboratory variables were reliable indicators for an early diagnosis: these were decreased platelet and white blood cell counts; decreased levels of albumin and fibrinogen; increased levels of ferritin, aspartate aminotransferase, and lactate dehydrogenase; and the presence of proteinuria. These indications provided the highest sensitivity and specificity necessary for an early diagnosis of MAS-associated SJIA 33.…”

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

confidence: 97%

“…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%

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

confidence: 90%

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

confidence: 97%

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

confidence: 99%

See 1 more Smart Citation

Macrophage activation syndrome: early diagnosis is key

Lerkvaleekul¹,

Vilaiyuk²

2018

OARRR

114

103

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“…Notably, most patients were asymptomatic and were diagnosed with MAS based on test results. TCZ inhibition of IL-6 activity may suppress clinical symptoms of MAS; therefore, patients should be carefully monitored for clinical symptoms and for changes in laboratory parameters, such as reduced platelet counts 23. Moreover, patients should be monitored for infections, especially viral infections, which are known risk factors for MAS24 and were suspected in seven of the MAS events reported.…”

Section: Discussionmentioning

confidence: 99%

Tocilizumab in systemic juvenile idiopathic arthritis in a real-world clinical setting: results from 1 year of postmarketing surveillance follow-up of 417 patients in Japan

et al. 2015

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ObjectivesTo evaluate the safety and effectiveness of tocilizumab (TCZ) in patients with systemic juvenile idiopathic arthritis (sJIA) in real-world clinical settings in Japan.MethodsPaediatric patients with sJIA initiating TCZ between April 2008 and February 2012 and those previously enrolled in clinical trials who initiated TCZ before April 2008 were enrolled in a Japanese registry surveillance programme. Safety and effectiveness parameters were collected for 52 weeks.ResultsOf 417 patients enrolled, mean age was 11.2 years and 48.0% were female. TCZ exposure was 407.0 patient-years (PYs). Baseline corticosteroid use was higher than in clinical trials. Rates of total adverse events (AEs) and serious AEs (SAEs) were 224.3/100 PYs and 54.5/100 PYs, respectively, with SAEs higher than previously reported. The most frequent AEs and SAEs were infections and infestations (69.8/100 PYs and 18.2/100 PYs, respectively). 74 serious infections occurred in 55 patients (18.2/100 PYs); higher than previously reported. 26 macrophage activation syndrome events were reported in 24 patients (6.4/100 PYs). Fever and rash symptoms improved from baseline to week 52 (54.6% to 5.6% and 43.0% to 5.6%, respectively). At 4 weeks, 8 weeks and 52 weeks, 90.5%, 96.2% and 99.0% of patients achieved normal C reactive protein levels (<0.3 mg/dL), respectively.ConclusionsThese first real-world data demonstrated that TCZ was well tolerated, with acceptable safety and effectiveness in patients with sJIA. Higher incidences of SAEs and serious infections may be due to differences, such as corticosteroid use and concomitant diseases, between patient populations enrolled in previously reported clinical trials and this study.

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“…Перечисленные диагностические трудности побудили нас к разработке критериев ранней диагностики САМ у па-циентов с СЮИА [27]. За основу были взяты только лабора-торные критерии как наиболее чувствительные и специфич-ные.…”

Section: фагоцитоз гемопоэтических клеток в км* гемофагоцитирующие мunclassified

Current approaches to diagnosing and treating macrophage activation syndrome in children with rheumatic diseases

Костик¹,

Дубко²,

Масалова³

et al. 2015

RJTAO

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Identification of the best cutoff points and clinical signs specific for early recognition of macrophage activation syndrome in active systemic juvenile idiopathic arthritis

Cited by 42 publications

References 28 publications

Macrophage activation syndrome: early diagnosis is key

Macrophage activation syndrome: early diagnosis is key

Tocilizumab in systemic juvenile idiopathic arthritis in a real-world clinical setting: results from 1 year of postmarketing surveillance follow-up of 417 patients in Japan

Current approaches to diagnosing and treating macrophage activation syndrome in children with rheumatic diseases

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