Differences and similarities of multisystem inflammatory syndrome in children, Kawasaki disease and macrophage activating syndrome due to systemic juvenile idiopathic arthritis: a comparative study

Abstract

To compare the clinical and laboratory findings of multisystem inflammatory syndrome in children (MIS-C), patients with Kawasaki disease (KD) and with macrophage activating syndrome due to systemic juvenile idiopathic arthritis (sJIA-MAS) on real-life data. Patients diagnosed with MIS-C, KD, and sJIA-MAS from 12 different centers in Turkey who were followed for at least 6 months were included in the study. Demographic, clinical, and laboratory findings of all patients were analyzed. A total of 154 MIS-C, 59 KD, and 31 sJIA-MAS patients were included. The median age of patients with MIS-C were higher than those with KD while lower than those with sJIA-MAS (8.2, 3, 12 years, respectively). Myalgia (39.6%), cardiac (50.6%), gastrointestinal (72.7%), and neurological (22.1%) involvements were more common in patients with MIS-C compared to others. MIS-C patients had lower levels of lymphocyte (950 vs 1700 cells/µl) and thrombocyte (173,000 vs 355,000 cells/µl) counts and higher pro-BNP (1108 vs 55 pg/ml) levels than KD. Ferritin levels were higher in patients with MIS-C compared to patients with KD while they were lower than patients with sJIA-MAS (440, 170, 10,442 ng/ml, respectively). Patients with MIS-C had a shorter duration of hospitalization than sJIA-MAS ( p = 0.02) while they required intensive care unit admission more frequently (55 vs 8 patients, p < 0.001). The median MAS/sJIA score of MIS-C patients was − 1.64 (− 5.23 to 9.68) and the median MAS/sJIA score of sJIA-MAS patients was −2.81 ([− 3.79] to [− 1.27]). MIS-C patients displayed certain differences in clinical and laboratory features when compared to KD and sJIA-MAS. Definition of the differences and similarities between MIS-C and the other intense inflammatory syndromes of childhood such as KD and MAS will help the clinicians while making timely diagnosis.

Distinguishing Between Multisystem Inflammatory Syndrome, Associated With COVID-19 in Children and the Kawasaki Disease: Development of Preliminary Criteria Based on the Data of the Retrospective Multicenter Cohort Study

“…Diversity between the two illnesses is also obvious as there are signs and symptoms with high frequency in MIS-C which are less commonly or rarely presented in KD patients. For example, gastrointestinal symptoms are more common for MIS-C according to many studies including our observation ( 11 , 22 , 29 ). It should also be noted that among the studied patients with gastrointestinal disorders in our MIS-C group, one had acute appendicitis, which required laparoscopic appendectomy, and the “white” appendix was removed.…”

“…HScore >105 was associated with the severest signs of MIS-C: myocarditis [OR = 4.2 (95% CI: 1.5, HScore is a simple tool which might be used for assessment of severity of MIS-C. Additionally, there is a difference in platelet levels in MIS-C and KD patients in our study, with median of 185 and 520 * 10 9 /l, respectively. Thrombocytosis is typical for KD, but less common in MIS-C patients, who quite often have a tendency toward thrombocytopenia, especially at onset or during the peak of the disease (8,20,22,29). Additionally, elevated biochemical markers of cardiac injury and highly elevated ferritin and D-dimer are typical for MIS-C patients (20,22,29).…”

Hematuria as an Early Sign of Multisystem Inflammatory Syndrome in Children: A Case Report of a Boy With Multiple Comorbidities and Review of Literature

“…Evidence supporting this theory incorporates the resemblance of MIS-C and Kawasaki disease, a known vasculitis ( 35 – 37 ). Similar features of these two inflammatory disorders include prolonged fever, increased inflammatory markers, rash, non-exudative conjunctivitis, and mucous involvement (cracked lips, strawberry tongue) ( 33 , 35 – 37 ). Despite these resemblances, there are also marked differences in clinical manifestations, such as older age of onset, more frequently observed gastrointestinal involvement, and a more severe disease course in MIS-C, as well as dissimilarities in laboratory findings, such as lymphopenia, thrombocytopenia, and elevated D-dimer levels in MIS-C ( 33 , 35 , 37 ).…”

“…Similar features of these two inflammatory disorders include prolonged fever, increased inflammatory markers, rash, non-exudative conjunctivitis, and mucous involvement (cracked lips, strawberry tongue) ( 33 , 35 – 37 ). Despite these resemblances, there are also marked differences in clinical manifestations, such as older age of onset, more frequently observed gastrointestinal involvement, and a more severe disease course in MIS-C, as well as dissimilarities in laboratory findings, such as lymphopenia, thrombocytopenia, and elevated D-dimer levels in MIS-C ( 33 , 35 , 37 ). Comparison of inflammatory cells and 180 plasma proteins in patients with MIS-C and Kawasaki disease revealed that both conditions have elements of hyperinflammation and vasculitis but a different cytokine pattern, suggesting different pathogenesis ( 33 ).…”

“…However, evidence is mounting that vasculopathy has an important role in MIS-C (33,34). Evidence supporting this theory incorporates the resemblance of MIS-C and Kawasaki disease, a known vasculitis (35)(36)(37). Similar features of these two inflammatory disorders include prolonged fever, increased inflammatory markers, rash, non-exudative conjunctivitis, and mucous involvement (cracked lips, strawberry tongue) (33,(35)(36)(37).…”