scholarly journals Importance of Serum Ferritin Level for Early Diagnosis and Differentiation in Patients with Kawasaki Disease with Macrophage Activation Syndrome

Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 269
Author(s):  
Da Eun Roh ◽  
Jung Eun Kwon ◽  
Hee Joung Choi ◽  
Yeo Hyang Kim
Keyword(s):  
Kawasaki Disease ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Screening Test ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Early Screening ◽  
Activation Syndrome

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.

scholarly journals Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease

2021 ◽  
Vol 11 (1) ◽  
pp. 206
Author(s):  
Clément Javaux ◽  
Thomas El-Jammal ◽  
Pierre-Antoine Neau ◽  
Nicolas Fournier ◽  
Mathieu Gerfaud-Valentin ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Predictive Factors ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Ferritin Level ◽  
Neurological Symptoms ◽  
Still’S Disease ◽  
Still's Disease ◽  
Specific Factors ◽  
Activation Syndrome

Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still’s disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14–11.2; p = 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08–15.3; p = 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 μg/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (p < 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS.

Intensive care requirement, rather than degree of serum ferritin elevation, predicts mortality in macrophage activation syndrome

2012 ◽  
Vol 13 (5) ◽  
pp. 616-617 ◽  
Author(s):  
Tellen D. Bennett ◽  
Kristen N. Hayward ◽  
Sarah Ringold ◽  
Carol A. Wallace ◽  
Reid W.D. Farris ◽  
...  
Keyword(s):  
Intensive Care ◽  
Serum Ferritin ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Care Requirement ◽  
Activation Syndrome

scholarly journals Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Masaki Shimizu ◽  
Mao Mizuta ◽  
Nami Okamoto ◽  
Takahiro Yasumi ◽  
Naomi Iwata ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Clinical Features ◽  
Early Treatment ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Classification Criteria ◽  
Real Patient ◽  
Laboratory Features ◽  
Activation Syndrome

Abstract Background This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ. Methods A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS. Results Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients. Conclusion TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIA patients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.

Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease

2010 ◽  
Vol 17 (2) ◽  
pp. 177 ◽  
Author(s):  
Hyoun-Jin Park ◽  
Yoon-Jeong Cho ◽  
E-Young Bae ◽  
Ui-Yoon Choi ◽  
Soo-Young Lee ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Extreme Form ◽  
Activation Syndrome

scholarly journals AB1044 CYTOKINE PROFILE OF MACROPHAGE ACTIVATION SYNDROME ASSOCIATED WITH KAWASAKI DISEASE

2019 ◽  
Author(s):  
Naoto Sakumura ◽  
Masaki Shimizu ◽  
Asumi Jinkawa ◽  
Hitoshi Irabu ◽  
Maiko Takakuwa ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Cytokine Profile ◽  
Activation Syndrome

scholarly journals High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

2019 ◽  
Vol 12 (8) ◽  
pp. e229708 ◽  
Author(s):  
Marie Lind-Holst ◽  
Ulla Birgitte Hartling ◽  
Anne Estmann Christensen
Keyword(s):  
Pericardial Effusion ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Additional Treatment ◽  
High Dose ◽  
Acute Phase Reactants ◽  
High Dose Methylprednisolone ◽  
Activation Syndrome

We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

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