A Case of Complete Adult-Onset Kawasaki Disease: A Review of Pathogenesis and Classification

Abstract

Kawasaki disease (KD) is an acute systemic vasculitis that occurs primarily in children and rarely in adults, possibly after bacterial or viral infections in genetically susceptible hosts. KD may frequently be undiagnosed especially in adult patients without the presence of all the classical clinical criteria (incomplete or atypical KD). In addition, many differential diagnoses could be considered. Here, we report a case of KD in an adult patient with clinical features characteristic of the classical form. KD requires a long-term management in both paediatric and adult patients, in order to avoid complications that could follow both the acute and retrospective diagnoses of KD.

Kawasaki disease in adults: Observations in France and literature review

“…We excluded cases with 1) drug hypersensitivity, staphylococcal scalded skin syndrome, infectious disease, inflammatory and autoimmune disease that could explain all or some of the clinical manifestations [8]; 2) unknown HIV serology status; 3) onset of some clinical findings before age 18; and 4) incomplete cases without coronary artery disease.…”

“…The total reported cases of AKD in the PubMed database represents 41 non-French complete cases [6,8, and 5 incomplete cases [102][103][104][105][106]. Some reports published between 1976 and 1996 were inaccessible (n=10) [53][54][55][56][57][58][59][60][61][62].…”

“…Given the exceptional nature and the need to rule out many differential diagnoses, the diagnosis of AKD is late and probably underdiagnosed. The differential diagnosis of AKD include: drug hypersensitivity reactions, toxic shock syndrome, erythema multiforme, scarlet fever, measles, rubella, parvovirus, infectious mononucleosis, hand-foot-and-mouth syndrome, leptospirosis, rocky mountain and Mediterranean spotted fever, syphilis, endocarditis, rheumatic fever, Reiter syndrome, palmoplantar psoriasis, Behçet disease, polyarteritis nodosa, Bazex syndrome, etc [8,33]. Hence, the diagnostic delay is longer for AKD than CKD, for which it is usually <7 days [7].…”

An adult presentation consistent with PIMS-TS

“…Although it is well known that Kawasaki disease in children aged 1 year and younger has a worse prognosis than in older children (aged >1 year), particularly in boys with coronary artery disease, the severity of disease in this cohort is unusual compared with the literature, in which 10-20% of infants develop aneurysms. 4 We cannot link these cases to the COVID-19 pandemic, as serology and PCR results for SARS-CoV-2 were mostly negative. Nevertheless, we feel that it is important that paediatricians consider early aggressive treatment and close cardiac monitoring of Kawasaki disease in infants, in whom a severe disease course might be occurring.…”

“…Alternatively, cytokine release syndrome secondary to chimeric antigen receptor T-cell therapy has been linked to high IFN-γ release, whereas the involvement of the IL-1 family cytokines seemed less prominent. 4 Lastly, it is note worthy that cytokine storm has attracted great interest, as a result of its description as a major determinant of COVID-19 outcomes. Yet, despite there being no definitive understanding of its immunopathology, a hallmark of COVID-19-associated cytokine storms seems to be prominent IL-6 elevation, with only 25% of patients showing evidence for IL-1-driven macrophage activation or haemophagocytosis.…”