Paediatric inflammatory multisystem syndrome (PIMS-TS): a novel condition

ABSTRACT

Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection (PIMS-TS) is a novel condition which has recently emerged during the COVID-19 pandemic, consisting of persistent fever, inflammation, and evidence of single- or multiorgan dysfunction, with additional features where any other microbial causes has been excluded (https//www.rcpch.ac.uk/resources/paediatric-multisysteminflammatory- syndrome-temporally-associated-covid-19-pimsguidance). A 16-year-old healthy male presented with a 48-h history of headache, fever, diarrhoea, vomiting and a widespread rash. One month prior he had contracted SARS-CoV-2 with no complications. On examination there was a maculopapular rash across the trunk and buttocks with petechiae present on the lower limbs and flexural folds in addition to an erythematous rash across the nasal dorsum and cheeks. Blood tests revealed lymphopaenia, thrombocytopenia, hypertriglyceridaemia and raised C-reactive protein and ferritin. Autoimmune screen and SARS-CoV-2 polymerase chain reaction tests were negative. A skin biopsy revealed features of a SARS-CoV-2 related urticarial reaction pattern. The patient was treated for myocarditis, fulfilling the diagnosis of PIMS-TS. He was transferred to a specialist intensive care unit and treated with intravenous immunoglobulins and steroids, infliximab, aspirin and topical steroids. He is currently undergoing investigations for encephalitis post admission. PIMS-TS is a rare syndrome that shares features with Kawasaki disease, toxic shock syndrome, macrophage activation syndrome and bacterial sepsis. Haemophagocytic lymphohistiocytosis has also been linked to SARS-CoV-2 (Retamozo S, Brito-Zerón P, Sisó- Almirall A et al. Haemophagocytic syndrome and COVID-19. Clin Rheumatol 2021;40 1233-44). Clinicians should consider PIMS-TS as a differential in any child presenting with a fever, rash and evidence of systemic inflammation. Early recognition, involvement of a multidisciplinary team and prompt referral to critical care is essential in managing this life-threatening condition.