Under-Recognized Macrophage Activation Syndrome in Refractory Kawasaki Disease
A Wolf in Sheep’s Clothing
Bibliographic Data
| ID | 15713335 |
|---|---|
| Authors | Sangwon Rhee (0000-0002-0896-1442, Bucheon St. Mary's Hospital), Danbi Kim (0009-0003-2594-4999, Catholic University of Korea), Kyoungsoon Cho (0000-0002-0212-0992, Bucheon St. Mary's Hospital), Jung Woo Rhim (Catholic University of Korea), Soo Young Lee (0000-0002-2590-3084, Bucheon St. Mary's Hospital, corresponding author), Soo-Young Lee (0000-0003-0776-5084, Department of Pediatrics, Bucheon St. Mary’s Hospital, The Catholic University of Korea, Bucheon 14647, Korea), Dae Chul Jeong (0000-0003-0934-817X, The Catholic University of Korea Seoul St. Mary's Hospital) |
| Year | 2022 |
| Volume | 9 |
| Issue | 10 |
| Pages | 1588-1588 |
| Publication date | 2022-10-20 |
| Open Access | Yes |
| Type | ARTICLE |
| Venue | Children (JOURNAL) |
| Journal identifiers | ISSN: 2227-9067 • E-ISSN: 2227-9067 |
| Publisher | Multidisciplinary Digital Publishing Institute (PUBLISHER • CH) |
| DOI | 10.3390/children9101588 |
| PMID | 36291524 |
| OpenAlex | W4306916590 |
| Language | EN |
| Citations received | 1 |
| References cited | 28 |
Recognition of macrophage activation syndrome (MAS) in patients with refractory Kawasaki disease (KD) can be challenging. This study aimed to investigate the incidence of MAS in patients with refractory KD and to compare the characteristics of refractory KD and MAS. Medical records of 468 patients diagnosed with KD from January 2010 to December 2019 were retrospectively reviewed. Of the 468 KD patients, 63 were enrolled in the study as a refractory KD group ( n = 59) and an MAS group ( n = 4). The incidence of MAS was 0.8% (4/468) in patients with KD and 6.3% (4/63) in patients with refractory KD. Compared to the refractory KD group, the MAS group had higher frequencies of incomplete KD, hepatosplenomegaly, third-line treatment, and MAS screening, and showed lower levels of albumin. No significant differences were found in other clinical and laboratory findings. In addition to four patients with MAS, five patients with refractory KD who received third-line treatment showed severe systemic inflammation and organ dysfunction, but only one in five patients underwent MAS screening, including ferritin levels. In conclusion, given the relatively high incidence of MAS in children with refractory KD and the similar phenotype between refractory KD and MAS, we propose that MAS screening should be included in routine laboratory tests for refractory KD
Biology · Disease · Incidence (geometry · Kawasaki disease · Macrophage Activation Syndrome · Refractory (planetary science · Autoimmune and Inflammatory Disorders Research · Disability Education and Employment · Kawasaki Disease and Coronary Complications · Medicine · Gastroenterology · Internal Medicine
| Citations per year | 0.33 |
|---|---|
| Citation span | 2023 - 2023 (1) |
| Highly cited | No |