Kawasaki Disease-like Syndrome Research Articles

Characteristics of pediatric multi-system inflammatory syndrome (PMIS) associated with COVID-19: a meta-analysis and insights into pathogenesis

ObjectivesThere was an outbreak of pediatric multisystem inflammation syndrome (PMIS) was observed in multiple countries recently, and this syndrome was suspected to be associated with SARS-CoV-2 infection. At present, there is still no standardized diagnostic criteria and treatment regimen for PMIS, while the etiology and pathogenesis still remain unclear.MethodsWe performed a systematic review on PubMed and Embase from the time of inception to June 24th 2020 in order to find relevant cases.ResultsThere are seven studies included, and 80% of patients suffered persistent fever and 90% appeared gastrointestinal symptoms. IgG antibody against SARS-CoV-2 was positive on 81% of patients, while 37% of the patients were nucleic acid positive. C-reactive protein, IL-6 and PCT were elevated and intravenous immunoglobulin was a routine treatment for PMIS. There were more than half of patients required inotropic supports and mechanical ventilation were applied to 33% of patients. The median length of hospital stay was 10.66 days and 74% had admitted to accept intensive care.ConclusionsOur study documented three common types of PMIS clinical presentation: persistent fever and gastrointestinal symptoms, shocked with heart dysfunction and Kawasaki disease-like syndrome. PMIS patients proved with a marked inflammatory state were possibly associated with SARS-CoV-2 infection.

Distinguishing between typical Kawasaki disease and multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2

In recent months, there are increasing reports of a Kawasaki disease-like syndrome in children infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), termed ‘Paediatric Multisystem Inflammatory Syndrome temporally associated with SARS-CoV-2 (PIMS-TS)’ in the UK. Debate is ongoing regarding the nature of these pro-inflammatory syndromes. We herein propose that the platelet count may, at least in part, be able to help us differentiate between the two aforementioned syndromes. In a recent report, compared to a historical ‘classical’ Kawasaki disease (KD) cohort, patients with PIMS-TS had significantly lower platelet counts (188 vs 383 g/L, p < 0.0001). A possible explanation for this is their difference in underlying immunopathogenesis. In KD, the fundamental pathogenesis is thought to be immune complex-mediated, hence, the use of intravenous immunoglobulin (IVIg) which competes with the immunoglobulin Fc receptors (FcRs) on inflammatory cells, preventing the activation of these cells and thereby ameliorating the inflammatory response. If left untreated, these immune complexes activates the inflammatory cells (including monocytes and neutrophils), which also results in recruitment of platelets, resulting in the thrombocytosis we commonly see in KD. These immune complexes may also bind to platelets directly via FcRs on platelet membranes. In contrast, in viral-associated hyperinflammatory syndromes (e.g. PIMS-TS or MIS-C), there are mediators being secreted in the process of eradication of the virus (mainly to stimulate CD8+ cells to kill viral infected cells), which would inadvertently suppress bone marrow function and activate platelets, culminating in thrombocytopenia.

Endothelial dysfunction in COVID-19: a position paper of the ESC Working Group for Atherosclerosis and Vascular Biology, and the ESC Council of Basic Cardiovascular Science.

The COVID-19 pandemic is an unprecedented healthcare emergency causing mortality and illness across the world. Although primarily affecting the lungs, the SARS-CoV-2 virus also affects the cardiovascular system. In addition to cardiac effects, e.g. myocarditis, arrhythmias, and myocardial damage, the vasculature is affected in COVID-19, both directly by the SARS-CoV-2 virus, and indirectly as a result of a systemic inflammatory cytokine storm. This includes the role of the vascular endothelium in the recruitment of inflammatory leucocytes where they contribute to tissue damage and cytokine release, which are key drivers of acute respiratory distress syndrome (ARDS), in disseminated intravascular coagulation, and cardiovascular complications in COVID-19. There is also evidence linking endothelial cells (ECs) to SARS-CoV-2 infection including: (i) the expression and function of its receptor angiotensin-converting enzyme 2 (ACE2) in the vasculature; (ii) the prevalence of a Kawasaki disease-like syndrome (vasculitis) in COVID-19; and (iii) evidence of EC infection with SARS-CoV-2 in patients with fatal COVID-19. Here, the Working Group on Atherosclerosis and Vascular Biology together with the Council of Basic Cardiovascular Science of the European Society of Cardiology provide a Position Statement on the importance of the endothelium in the underlying pathophysiology behind the clinical presentation in COVID-19 and identify key questions for future research to address. We propose that endothelial biomarkers and tests of function (e.g. flow-mediated dilatation) should be evaluated for their usefulness in the risk stratification of COVID-19 patients. A better understanding of the effects of SARS-CoV-2 on endothelial biology in both the micro- and macrovasculature is required, and endothelial function testing should be considered in the follow-up of convalescent COVID-19 patients for early detection of long-term cardiovascular complications.

Fever and Erythema in the Emergency Room

The emergency department presents difficult cases for the consulting dermatologist. One of the most challenging is the patient who presents with fever and an erythematous rash. The differential diagnosis of the erythrodermic patient in the emergency room can include drug eruptions (DRESS syndrome), Staphylococcal scalded skin syndrome, toxic shock syndrome, Kawasaki disease and Kawasaki disease-like Syndrome, and recalcitrant erythematous desquamating disorder. Evaluating these patients can be frustrating and requires patience and thoroughness to arrive at the correct diagnosis.

Atypical kawasaki disease (KD) in an HIV-infected adolescent

Rationale Kawasaki disease-like syndrome (KDLS) has been described in HIV-infected individuals. However, coronary artery abnormalities have not been reported in these patients. Methods A 14-year-old boy with advanced perinatal HIV infection and ITP required hospital admission for fever and bilateral hand swelling of 3 days duration. Generalized erythematous rash, palmar erythema, and conjunctivitis were noted on examination. Blood, urine, and throat cultures were negative. HIV-1 RNA was 990,000 copies/mL and CD4 count was 10 cells/mm 3 (1%). All medications including didanosine, stavudine, tenofovir, enfuvirtide, azithromycin, and atovaquone were discontinued for a suspected drug reaction. High fever (Tmax 104.7°F) persisted despite 5 days of intravenous nafcillin and cefotaxime. Baseline echocardiogram revealed normal coronary anatomy. A diagnosis of presumed KDLS was made. Intravenous gamma globulin (2g/kg) was administered with resolution of patient's fever and conjunctivitis within 24 hours. Aspirin therapy was not initiated secondary to ITP. Results Three weeks later, ectasia of multiple coronary arteries (measuring up to 5.0 mm) was seen on echocardiogram. Desquamation was noted. One month later, a proximal saccular aneurysm of the left anterior descending artery was seen. Four months after presentation, the coronary aneurysms resolved. The patient remains well without recurrence of symptoms. Conclusions We report a case of atypical KD with coronary artery aneurysm formation in an HIV-infected adolescent. The development of KD in an extremely immunocompromised patient with ITP and lymphocytopenia implicates infection, rather than lymphocyte-dependent processes, in the pathogenesis of KD.