Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Ravelli, Angelo; Magni‐Manzoni, Silvia; Pistorio, Angela; Besana, C; Foti, T M; Ruperto, Nicolino; Viola, Stefania; Martini, Alberto

doi:10.1016/j.jpeds.2004.12.016

Cited by 371 publications

(313 citation statements)

References 27 publications

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“…The demonstration of macrophage hemophagocytosis in the bone marrow aspirate is required only in doubtful cases. As noted in patients with HLH 18 and in several instances of MAS, 17 the bone marrow aspirate does not always show hemophagocytosis, and, furthermore, hemophagocytosis is not always demonstrable in the initial stages of the disease. Repeat bone marrow aspirate over time may eventually demonstrate hemophagocytosis.…”

Section: Introductionmentioning

confidence: 88%

“…17 Laboratory criteria include decreased platelet count (p262 Â 10 9 l À1 ), elevated levels of aspartate aminotransferase (459 U l À1 ), decreased white blood cell count (p4.0 Â 10 9 l À1 ) and hypofibrinogenemia (p2.5 g l À1 ). Clinical criteria include hepatomegaly, hemorrhagic manifestations and central nervous system dysfunction.…”

Section: Introductionmentioning

confidence: 99%

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Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

et al. 2012

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Macrophage activation syndrome (MAS) is a severe, frequently fatal complication of systemic juvenile idiopathic arthritis (sJIA) with features of hemophagocytosis leading to coagulopathy, pancytopenia, and liver and central nervous system dysfunction. MAS is overt in 10% of children with sJIA but occurs subclinically in another 30 --40%. It is difficult to distinguish sJIA disease flare from MAS. Development of criteria for establishing MAS as part of sJIA are under way and will hopefully prove sensitive and specific. Mutations in cytolytic pathway genes are increasingly being recognized in children who develop MAS as part of sJIA. Identification of these mutations may someday assist in MAS diagnosis. Defects in cytolytic genes have provided murine models of MAS to study pathophysiology and treatment. Recently, the first mouse model of MAS not requiring infection but rather dependent on repeated stimulation through Toll-like receptors was reported. This provides a model of MAS that may more accurately reflect MAS pathology in the setting of autoinflammation or autoimmunity. This model confirms the importance of a balance between pro-and anti-inflammatory cytokines. There has been remarkable progress in the use of anti-pro-inflammatory cytokine therapy, particularly against interleukin-1, in the treatment of secondary forms of MAS, such as in sJIA.

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Section: Introductionmentioning

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

et al. 2012

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“…AOSDassociated MAS was diagnosed according to the criteria proposed by Imashuku et al [24]. s-JIA-associated MAS was diagnosed according to the criteria proposed by Ravelli et al [25]. The following criteria were used to define the active phase of s-JIA: active arthritis, fever, rash, hepatosplenomegaly, generalized lymphadenopathy, and serositis, as well as an increased erythrocyte sedimentation rate and C-reactive protein (CRP) level.…”

Section: Patients and Samplesmentioning

confidence: 99%

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

Inoue

Shimizu

Tsunoda

et al. 2016

Clinical Immunology

123

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To compare pro-inflammatory cytokine profiles and kinetics in patients with adult-onset Still's disease (AOSD) to those in patients with systemic juvenile idiopathic arthritis (s-JIA), we analyzed serum cytokine concentrations in 33 patients with AOSD and 77 patients with s-JIA and compared them with clinical features. Patients with AOSD and s-JIA shared a common cytokine profile pattern of a significant increase in IL-18. Patients with AOSD were classified into two subgroups based on serum IL-6 and IL-18 levels. The number of patients with arthritis was significantly higher in the IL-6-dominant subgroup. The cytokine patterns associated with s-JIA and AOSD share common features, such as a significant and predominant increase in IL-18. Distinct IL-6-and IL-18-based cytokine profiles might be responsible for distinct clinical manifestations. The presence of two distinct subgroups in patients with both diseases further supports the view that s-JIA and AOSD share a disease category.

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“…При этом заболевании основными лабораторными изменениями яв-ляются высокий уровень лейкоцитов, тромбоцитов, фибриноге-на и СОЭ. Снижение данных параметров, исходно повышенных (даже без достижения патологически низкого уровня), уже может свидетельствовать о развивающемся ГФС [10]. Как правило, ци-топения и гипофибриногенемия у пациентов с БСВ и ювениль-ным РА выявляется на поздних стадиях, когда лечение становит-ся малоэффективным [11].…”

Section: диагностические критерии гфс 2004 г (диагноз при 5 из 8 криunclassified

“…Однако на ранней стадии развития заболевания гемо-фагоцитоз может отсутствовать, а рекомендуемое как наиболее доступное исследование КМ в таком случае окажется неинфор-мативным [10]. Выявление гемофагоцитов в ткани печени также в соответствии с критериями позволяет подтвердить диагноз ГФС [14].…”

Section: диагностические критерии гфс 2004 г (диагноз при 5 из 8 криunclassified

Hemophagocytic syndrome in patients with adult-onset Still’s disease: Diagnostic problems

Zakharova¹,

Mutovina²,

Гордеев³

et al. 2015

Ter. arkh.

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Болезнь Стилла взрослых (БСВ) -редкое системное воспа-лительное заболевание неизвестной этиологии, характеризую-щееся продолжительной фебрильной лихорадкой, нейтрофиль-ным лейкоцитозом, нестойкой полиморфной сыпью, полиартри-том и мультиорганным поражением [1]. В большинстве случаев прогноз БСВ благоприятный. Однако при БСВ возможно разви-тие потенциально фатального осложнения -гемофагоцитарного синдрома (ГФС), также именуемого гемофагоцитарным лимфо-гистиоцитозом, или синдромом активации макрофагов, обуслов-ленного массивным выбросом цитокинов из активированных лимфоцитов и макрофагов («цитокиновый шторм»). ЗАМЕТКИ ИЗ ПРАКТИКИ АннотацияГемофагоцитарный синдром (ГФС) -редкое угрожающее жизни состояние, обусловленное массивным выбросом цитокинов из активированных макрофагов и лимфоцитов. Описано развитие ГФС при различных заболеваниях инфекционного, злокачественного и аутоиммунного характера. Приводится описание клинического случая развития гемофагоцитарного синдрома у 63-летней пациентки с болезнью Стилла взрослых с фебрильной лихорадкой, сопровождавшейся нейтрофильным лейкоцитозом и резким сдвигом лейкоцитарной формулы влево, высоким уровнем прокальцитонина, гепатоспленомегалией, отечным синдромом и прогрессирующей полиорганной недостаточностью с развитием синдрома диссеминированного внутрисосудистого свертывания и респираторного дистресс-синдрома взрослых. Диагноз ГФС установлен в соответствии с диагностическими критериями и подтвержден результатами аутопсии -обнаружены фагоциты в тканях печени, легких. Ключевые слова: гемофагоцитарный синдром, болезнь Стилла взрослых, диагностические критерии.Hemophagocytic syndrome (HPS) is a rare life-threatening condition caused by massive cytokine release from activated macrophages and lymphocytes. The paper depicts the development of HPS in different infections, malignancies, and autoimmune diseases. It describes a clinical case of hemophagocytic syndrome in a 63-year-old female patient with adult-onset Still's disease and high fever accompanied by neutrophil leukocytosis and a drastic left leukocyte count shift, high procalcitonin levels, hepatosplenomegaly, edematous syndrome, and progressive multiple organ dysfunction with the development of disseminated intravascular coagulation and adult respiratory distress syndrome. The diagnosis of HPS was established according to the diagnostic criteria and verified by autopsy: phagocytes in liver and lung tissues.

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Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Cited by 371 publications

References 27 publications

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Cytokine profile in adult-onset Still's disease: Comparison with systemic juvenile idiopathic arthritis

Hemophagocytic syndrome in patients with adult-onset Still’s disease: Diagnostic problems

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