SARS-CoV-2–related MIS-C: A key to the viral and genetic causes of Kawasaki disease?
Authors: Vanessa Sancho-Shimizu, Petter Brodin, Aurélie Cobat, Catherine M. Biggs, Julie Toubiana, Carrie L. Lucas, Sarah E. Henrickson, Alexandre Belot, Elie Haddad, Kathie Beland, Aurora Pujol, Agatha Schlüter, Laura Planas-Serra, Sergio Aguilera-Albesa, Juan Valencia-Ramos, Agustí Rodríguez-Palmero, Marta Gut, Jacques G. Rivière, Roger Colobran, Pere Soler-Palacin, Carlos Rodriguez-Gallego, Rebeca Perez De Diego, Carlos Flores, Laia Alsina, Daniel Blazquez-Gamero, Iolanda Jordan, Sevgi Keles, Melike Emiroglu, Ozge Metin Akcan, Gulsum Alkan, Selma Erol Aytekin, Yahya Gul, Şadiye Kübra Tüter Öz, Sefika Elmas Bozdemir, Gulsum Iclal Bayhan, Saliha Kanık-Yüksek, Aslınur Özkaya Parlakay, Belgin Gülhan, Aysun Yahşi, Ahmet Osman Kilic, Adem Karbuz, Emine Hafize Erdeniz, Esra Akyüz Özkan, Zerrin Orbak, Şehnaz Aydemir, Jale Bengi Celik, Bahar Kandemir, Gökhan Aytekin, Hasan Kapakli, Volkan Yarar, Alper Yosunkaya, Hulya Vatansev, Caner Aytekin, Selda Hancerli Torun, Serdar Nepesov, Taner Coskuner, Betül Sözeri, Yasemin Kendir Demirkol, Ozgur Kasapcopur, Mehmet Yıldız, Esra Sevketoglu, Nevin Hatipoğlu, Tayfun Özçelik, Osman Yesilbas, Zeynep Gökçe Gayretli Aydin, Anna Sediva, Adam Klocperk, Marketa Bloomfield, Isabelle Meyts, Selket Delafontaine, Filomeen Haerynck, Levi Hoste, Mohammad Shahrooei, Laura Marque, João Farela Neves, Giuseppe Novelli, Antonio Novelli, Alessandro Aiuti, Giorgio Casari, Amed Aziz Bousfiha, Saleh Zaid Almuhsen, Ali Sobh, Alenka Gagro, Fanny Bajolle, Damien Bonnet, Pierre Lebon, Weite Lei, Danyel Lee, Yoann Seeleuthner, Peng Zhang, Majistor Maglorius, Quentin Philippot, Simon Pelham, Paul Bastard, Qian Zhang, Emmanuelle Jouanguy, Anne Puel, Jethro Herberg, Taco W Kuijpers, Evangelos Bellos, Myrsini Kaforou, Stephanie Menikou, Qiang Pan-Hammarström, Lennart Hammarström, Hassan Abolhassani, Yenan Bryceson, Antonio Condino-Neto, Carolina Prando, Silvia Yumi Bando, Andre Cavalcanti, Jacques Fellay, Geraldine Blanchard-Rohner, Davood Mansouri, Shima Mahmoudi, Oksana Boyarchuk, Alla Volokha, Anastasiia Bondarenko, Yuriy Stepanovskiy, Trine Mogensen, Diederik van de Beek, Evangelos Andreakos, Maria Papadaki, Ahmad Abou Tayoun, Rabih Halwani, Fahd Al-Mulla, José Luis Franco, Yu-Lung Lau, Mike Kwan, Kohsuke Imai, Satoshi Okada, Alexandre Bolze, Manish J. Butte, Elena Hsieh, Beth A Drolet, Lisa Arkin, Yuval Itan, Tom Maniatis, Moshe Arditi, Megan Cooper, Erica Schmitt, Samya Chakravorty, Mark S. Anderson, Helen C. Su, Luigi D. Notarangelo, Stuart G. Tangye, Joshua D. Milner, Michael Levin, Laurent Abel, Dusan Bogunovic, Jean-Laurent Casanova, Shen-Ying Zhang
Journal: The Journal of Experimental Medicine (2021)

Multisystem inflammatory syndrome in children (MIS-C) emerged in April 2020 in communities with high COVID-19 rates. This new condition is heterogenous but resembles Kawasaki disease (KD), a well-known but poorly understood and clinically heterogenous pediatric inflammatory condition for which weak associations have been found with a myriad of viral illnesses. Epidemiological data clearly indicate that SARS-CoV-2 is the trigger for MIS-C, which typically occurs about 1 mo after infection. These findings support the hypothesis of viral triggers for the various forms of classic KD. We further suggest that rare inborn errors of immunity (IEIs) altering the immune response to SARS-CoV-2 may underlie the pathogenesis of MIS-C in some children. The discovery of monogenic IEIs underlying MIS-C would shed light on its pathogenesis, paving the way for a new genetic approach to classic KD, revisited as a heterogeneous collection of IEIs to viruses.