Fri, 05/15/2020 | News

COVID-19, Kawasaki Disease (KD) and PIMS-TS in children

The Paediatric Active Enhanced Disease Surveillance (PAEDS) network, in conjunction with the Acute Inflammatory Vasculitis Working Group that currently conducts surveillance for Kawasaki Disease (KD) across Australia, has recently provided expert advice to the Australian Health Protection Principal Committee (AHPPC) on a newly described condition being reported to be potentially linked to COVID-19 infection in children in the UK, Europe and the USA. The AHPPC released a statement on this on the 14 May 2020.

Read the full statement here

Provisionally named Paediatric Inflammatory Multisystem Syndrome Temporally associated with SARS-COV-2 (PIMS-TS) and now also known as Multisystem Inflammatory Syndrome in Children (MIS-C) in the USA, this condition resembles two other well-described, uncommon childhood illnesses: Kawasaki disease (KD) and Toxic Shock Syndrome (TSS). 

KD is relatively uncommon, mostly affecting children under the age of 5 years but can occur in older children. It is not known what causes KD and there is currently no diagnostic test, leaving doctors to diagnose the disease on the basis of clinical criteria (such as presence of fever, rash, swollen lymph nodes and red eyes). The most serious complication of KD is damage to the coronary arteries, potentially requiring long-term management. Rarely children can present critically unwell with shock (low blood pressure) due to impaired heart muscle function – known as Kawasaki shock syndrome (KSS), with overlapping features of toxic shock syndrome (TSS). 

Concerns have been raised that this newly described condition, PIMS-TS, may be linked to KD or TSS, as approximately 25% of PIMS-TS cases reported to date have evidence of damage to the coronary arteries. However, there are some significant differences: PIMS-TS appears to affect older children, gastrointestinal symptoms (particularly abdominal pain) predominate and there appears to be a greater chance of damage to the heart and kidneys. While the majority of patients have tested positive for SARS-COV-2, these patients have not been infectious at the time of diagnosis and the exact link between this condition and SARS-CoV-2 remains unclear.

PIMS-TS/MIS-C has been reported in small numbers internationally in children from countries that are experiencing widespread community-based transmission of SARS-CoV-2 and therefore much higher rates of paediatric disease. To date, there have been no reported cases of PIMS-TS/MIS-C, KD or TSS in children associated with COVID-19 in Australia. Australia has had <150 cases of COVID-19 in children aged <15 years and therefore it is highly unlikely we will see any cases of PIMS-TS/MIS-C. 

Since 2019 our national sentinel surveillance system (PAEDS) has conducted national surveillance of Kawasaki Disease. Since the onset of the pandemic in 2020, PAEDS has not detected any increase in KD case numbers compared with those in previous years. If numbers of KD do rise in the coming months, we are confident that our existing surveillance mechanisms will identify this and be able to report it. PAEDS also partners with FluCAN to collect enhanced clinical data on all children presenting to our PAEDS sites with COVID-19. 

We are also leading development of surveillance for PIMS-TS/MIS-C in Australia, and will be collaborating with other networks, both nationally and internationally, to ensure any cases of PIMS-TS/MIS-C are rapidly detected and comprehensively investigated.

As with any serious paediatric condition, clinicians should follow recommended clinical management pathways for COVID-19, KD or TSS. For any patient with these conditions suspected to have PIMS-TS/MIS-C, it is important to ensure testing for SARS-CoV-2 by PCR on appropriate specimens and also collect a blood sample for testing of antibodies (serology) to SARS-CoV-2 prior to potential use of intravenous immunoglobulin (IVIG) therapy.

Paediatricians who are managing potential cases of PIMS-TS/MIS-C, KD or TSS should ensure they contact the relevant paediatric infectious diseases specialist in their state or territory, or (for KD specifically) a paediatric rheumatologist or immunologist. 

A list of all PAEDS sites that participate in surveillance for these conditions is available here, and specialists can be reached via the respective hospital switchboards.

Media contact: Madeliene Smith 0429 350 279 
For further information or enquiries email the PAEDS team (Monday – Friday)