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

• DOI: 10.1007/s00296-021-04980-7
• Publication Date: 2021-09-07T08:02:50Z
• Authors: Gülçin Otar Yener; Ayşenur Paç Kısaa...; Kadir Ulu; Erdal Atalay; Fatih Haşlak; Semanur Özdel; Burcu Bozkaya Yücel; Deniz Gezgin Yıld...; Figen Çakmak; Kübra Öztürk; Mustafa Çakan; Zeynep Balık; Canan Hasbal Akkuş; Mehmet Yıldız; Tuğba Erat; Benhur Şirvan Çetin; Münevver Yılmaz; Esra Bağlan; Sibel Laçinel Gür...; Vildan Atasayan; Şerife Gül Karadağ; Amra Adrovic; Şengül Çağlayan; Ayşe Tanatar; Fatma Gül Demirkan; Taner Coşkuner; Özlem Akgün; Müşerref Kasap Cü...; Gülşah Kavrul Kay...; Sezgin Şahin; Özge Başaran; Ferhat Demir; Kenan Barut; Murat Çiftel; Dolunay Gürses; Ali Baykan; Yasemin Özsürekçi; Tevfik Karagöz; Hafize Emine Sönmez; Yelda Bilginer; Nuray Aktay Ayaz; Özlem Aydoğ; Selçuk Yüksel; Betül Sözeri; Özgür Kasapçopur; Seza Özen
• Keywords: Macrophage Activation Syndrome

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.