Is Kawasaki Disease linked to Ehlers-Danlos Syndrome?

The connection between Kawasaki Disease (KD) and conditions like Ehlers-Danlos Syndrome (EDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS) has been explored, particularly in the context of more severe complications like aneurysms that might arise in some patients. However, the evidence remains speculative and not conclusive.

1. Ehlers-Danlos Syndrome (EDS): There is a hypothesis suggesting that some patients with severe Kawasaki or Kawasaki-like disease might have underlying EDS or related connective tissue disorders. The reasoning behind this includes shared symptoms like joint hypermobility, skin elasticity issues, and cardiovascular complications such as aneurysms​. However, this is still largely a hypothesis, and more research is needed to confirm any direct link.
2. POTS: POTS is often seen in patients with hypermobile EDS (hEDS). The connection here might be more about shared symptoms like dysautonomia rather than a direct link between POTS and KD​.
3. Mast Cell Activation Syndrome (MCAS): There is also speculation about the role of mast cell activation in both EDS and KD, particularly because of the shared inflammatory responses. Both conditions can involve an overactive immune response, which might explain some overlap in symptoms​​.
4. COVID-19 and Kawasaki-like Syndrome: During the COVID-19 pandemic, there were reports of a Kawasaki-like syndrome in children, known as Multisystem Inflammatory Syndrome in Children (MIS-C). This condition has drawn attention to whether children with underlying conditions like EDS might be at increased risk. However, no direct evidence currently supports a higher risk for severe complications from Kawasaki-like disease in children with EDS​​.

In summary, while there are interesting overlaps and hypotheses connecting KD with EDS, POTS, and MCAS, especially concerning severe complications, there is no strong or direct evidence confirming these links. The connections remain speculative and are areas of ongoing research.

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References:

1. Wilson, Golder N., and Vijay S. Tonk. "Do Severe Complications Like Aneurysms from Kawasaki and Kawasaki-Like Infections Arise in Children with Underlying Articulo-Autonomic Dysplasia/Ehlers-Danlos Syndrome?" Annals of Pediatric Research, vol. 4, no. 3, 2020, article 1042.
2. Kim, Gi Beom. "Reality of Kawasaki Disease Epidemiology." Korean Journal of Pediatrics, vol. 62, no. 8, 2019, pp. 292-296. doi:10.3345/kjp.2019.00157.
3. Welch, Thomas R. "Can We Identify Patients with Kawasaki Disease at Risk for Resistance to Immunoglobulin?" The Journal of Pediatrics, vol. 159, no. 5, 2011, pp. 826-831. doi:10.1016/j.jpeds.2011.02.035.

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