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. 2022 May;42(5):879-889.
doi: 10.1007/s00296-021-04980-7. Epub 2021 Sep 7.

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

Gülçin Otar Yener  1 , Ayşenur Paç Kısaarslan  2 , Kadir Ulu  3 , Erdal Atalay  4 , Fatih Haşlak  5 , Semanur Özdel  6 , Burcu Bozkaya Yücel  7 , Deniz Gezgin Yıldırım  8 , Figen Çakmak  9 , Kübra Öztürk  10 , Mustafa Çakan  11 , Zeynep Balık  4 , Canan Hasbal Akkuş  12 , Mehmet Yıldız  5 , Tuğba Erat  13 , Benhur Şirvan Çetin  14 , Münevver Yılmaz  15 , Esra Bağlan  6 , Sibel Laçinel Gürlevik  16 , Vildan Atasayan  17 , Şerife Gül Karadağ  18 , Amra Adrovic  5 , Şengül Çağlayan  3 , Ayşe Tanatar  9 , Fatma Gül Demirkan  9 , Taner Coşkuner  3 , Özlem Akgün  9 , Müşerref Kasap Cüceoğlu  4 , Gülşah Kavrul Kayaalp  9 , Sezgin Şahin  5 , Özge Başaran  4 , Ferhat Demir  3 , Kenan Barut  5 , Murat Çiftel  19 , Dolunay Gürses  15 , Ali Baykan  20 , Yasemin Özsürekçi  16 , Tevfik Karagöz  21 , Hafize Emine Sönmez  22 , Yelda Bilginer  4 , Nuray Aktay Ayaz  9 , Özlem Aydoğ  7 , Selçuk Yüksel  23 , Betül Sözeri  3 , Özgür Kasapçopur  5 , Seza Özen  4
Affiliations

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

Gülçin Otar Yener et al. Rheumatol Int. 2022 May.

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.

Keywords: Kawasaki disease (KD); Macrophage activation syndrome; Multisystem inflammatory syndrome in children (MIS-C); Systemic juvenile idiopathic arthritis.

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Conflict of interest statement

The authors declare no conflicts of interest.

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