Clinical Management
Loubna Lamrani, n/a
Medical student
McGill University
Montreal, Quebec, Canada
Background: Kawasaki Disease Shock Syndrome (KDSS) represents 1.2-7% of KD and could be misdiagnosed for other aetiologies. This retrospective multi-center study from 6 collaborating institutions (Montreal, Canada; Seattle, Washington, and San Francisco, USA; Mexico City, Mexico; and Chandigrah, India) aims to better understand diagnostic challenges, therapeutic management, and potential risk factors of outcome in contemporaneous cohort of KDSS patients.
Methods: We collected data on KDSS patients defined by hypotension or Intensive Care Unit (ICU) management between 1995 and 2019.
Results: Of 71 KDSS cases, 27 (39.7%) had incomplete KD criteria. Median age was 5.5 years [3-9] (1 case >10 years), 38 (53.5%) males. Diagnosis of KD was established 1 day [0-4] after admission, and 9 days [4.5-11] from onset of fever. There were 18 cases misdiagnosed at admission as septic shock, toxic shock syndrome, or other causes (Table-1). Incomplete KD criteria were more prevalent among the misdiagnosed cases in 8/18 (44%) compared to 9/53 (17%) with proper KDSS diagnosis (p < 0.0001). Multi-organ systems were involved in 26 (37%) cases (12.7%, 15.5%, and 8.5% with 2, 3, or >3 systems). Management did not require ICU in 15 (20%), whereas ICU was needed in 42/56 (75%) for advanced shock management, including Macrophage Activation Syndrome 1, and other causes for 13 cases, such as respiratory arrest, congestive heart failure, reaction to IVIG. The median ICU stay was 4 days [2-13], total hospitalisation 9 days [6-13]. Shock management included (Table 2) volume replacement 47 (68%), inotropic agents 29 (43%), vasoactive agents 22 (34%), and ventilatory support 21 (31%); multiple modalities were involved in 40 (58.1%) (Figure-1). IVIG was received by 95% of cases, with a resistance rate of 50% (38% received a 2nd dose, and 8.5% a 3rd dose). Adjuvant therapy was administered simultaneously with shock treatment to 11 (15.4%). Most patients, 66/71 (92.9%), were discharged, whereas 2 (2.8%) succumbed to the disease 1 day and 27 days after admission. After discharge, 3 (4.2%) were readmitted for recurrence of KD symptoms, but no relapse of shock.
Conclusion: Initial KDSS diagnosis was promptly established in general. Diagnostic delay was predominantly associated with an incomplete KD presentation. The ICU stay was short in general, required multiple modalities of advanced shock treatments, multiple doses of IVIG, and adjuvant anti-inflammatory drugs. Multi-organ system involvement is prevalent among this population and raises concerns about timely diagnosis, appropriate management guidelines, and further studies on this disease.