SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

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

doi:10.1084/jem.20210446

SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

, 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 HatipoğluTayfun 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

Research output: Contribution to journal › Review article › Research › peer-review

121 Citations (Scopus)

Abstract

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.

Original language	English
Article number	e20210446
Number of pages	24
Journal	Journal of Experimental Medicine
Volume	218
Issue number	6
DOIs	https://doi.org/10.1084/jem.20210446
Publication status	Published - 27 Apr 2021

Access to Document

10.1084/jem.20210446

Cite this

, Orbak, Z., Aydemir, Ş., Bengi Celik, J., Kandemir, B., Aytekin, G., Kapakli, H., Yarar, V., Yosunkaya, A., Vatansev, H., Aytekin, C., Hancerli Torun, S., Nepesov, S., Coskuner, T., Sözeri, B., Kendir Demirkol, Y., Kasapcopur, O., Yıldız, M., Sevketoglu, E., ... Notarangelo, L. D. (2021). SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease? Journal of Experimental Medicine, 218(6), Article e20210446. https://doi.org/10.1084/jem.20210446

@article{3a427534e3bd411089f0bf9c970dc268,

title = "SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease?",

abstract = "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.",

author = "Vanessa Sancho-Shimizu and Petter Brodin and Aur{\'e}lie Cobat and Biggs, {Catherine M.} and Julie Toubiana and Lucas, {Carrie L.} and Henrickson, {Sarah E.} and Alexandre Belot and Tangye, {Stuart G.} and Milner, {Joshua D.} and Michael Levin and Laurent Abel and Dusan Bogunovic and Casanova, {Jean Laurent} and Zhang, {Shen Ying} and Elie Haddad and Kathie Beland and Aurora Pujol and Agatha Schl{\"u}ter and Laura Planas-Serra and Sergio Aguilera-Albesa and Juan Valencia-Ramos and Agust{\'i} Rodr{\'i}guez-Palmero and Marta Gut and Rivi{\`e}re, {Jacques G.} and Roger Colobran and Pere Soler-Palacin and Carlos Rodriguez-Gallego and {de Diego}, {Rebeca Perez} and Carlos Flores and Laia Alsina and Daniel Blazquez-Gamero and Iolanda Jordan and Sevgi Keles and Melike Emiroglu and Akcan, {Ozge Metin} and Gulsum Alkan and Aytekin, {Selma Erol} and Yahya Gul and {T{\"u}ter {\"O}z}, {{\c S}adiye K{\"u}bra} and Bozdemir, {Sefika Elmas} and Bayhan, {Gulsum Iclal} and Y{\"u}ksek, {Saliha Kanık} and Parlakay, {Aslınur {\"O}zkaya} and Belgin G{\"u}lhan and Aysun Yah{\c s}i and Kilic, {Ahmet Osman} and Adem Karbuz and Erdeniz, {Emine Hafize} and {\"O}zkan, {Esra Aky{\"u}z} and Zerrin Orbak and {\c S}ehnaz Aydemir and {Bengi Celik,}, Jale and Bahar Kandemir and G{\"o}khan Aytekin and Hasan Kapakli and Volkan Yarar and Alper Yosunkaya and Hulya Vatansev and Caner Aytekin and {Hancerli Torun}, Selda and Serdar Nepesov and Taner Coskuner and Bet{\"u}l S{\"o}zeri and {Kendir Demirkol}, Yasemin and Ozgur Kasapcopur and Mehmet Yıldız and Esra Sevketoglu and Nevin Hatipoğlu and Tayfun {\"O}z{\c c}elik and Osman Yesilbas and {Gayretli Aydin}, {Zeynep G{\"o}k{\c c}e} and Anna Sediva and Adam Klocperk and Marketa Bloomfield and Isabelle Meyts and Selket Delafontaine and Filomeen Haerynck and Levi Hoste and Mohammad Shahrooei and Laura Marque and {Farela Neves}, Jo{\~a}o and Giuseppe Novelli and Antonio Novelli and Alessandro Aiuti and Giorgio Casari and {Aziz Bousfiha}, Amed and {Zaid Almuhsen}, Saleh and Ali Sobh and Alenka Gagro and Fanny Bajolle and Damien Bonnet and Pierre Lebon and Weite Lei and Danyel Lee and Yoann Seeleuthner and Peng Zhang and Majistor Maglorius and Quentin Philippot and Simon Pelham and Paul Bastard and Qian Zhang and Emmanuelle Jouanguy and Anne Puel and Jethro Herberg and Kuijpers, {Taco W} and Evangelos Bellos and Myrsini Kaforou and Stephanie Menikou and Qiang Pan-Hammarstr{\"o}m and Lennart Hammarstr{\"o}m and Hassan Abolhassani and Yenan Bryceson and Antonio Condino-Neto and Carolina Prando and {Yumi Bando}, Silvia and Andre Cavalcanti and Jacques Fellay and Geraldine Blanchard-Rohner and Davood Mansouri and Shima Mahmoudi and Oksana Boyarchuk and Alla Volokha and Anastasiia Bondarenko and Yuriy Stepanovskiy and Trine Mogensen and {van de Beek}, Diederik and Evangelos Andreakos and Maria Papadaki and {Abou Tayoun}, Ahmad and Rabih Halwani and Fahd Al-Mulla and Franco, {Jos{\'e} Luis} and Yu-Lung Lau and Mike Kwan and Kohsuke Imai and Satoshi Okada and Alexandre Bolze and Butte, {Manish J.} and Elena Hsieh and Drolet, {Beth A} and Lisa Arkin and Yuval Itan and Tom Maniatis and Moshe Arditi and Megan Cooper and Erica Schmitt and Samya Chakravorty and Anderson, {Mark S.} and Su, {Helen C.} and Notarangelo, {Luigi D.}",

note = "Publisher Copyright: {\textcopyright} 2021 Sancho-Shimizu et al.",

year = "2021",

month = apr,

day = "27",

doi = "10.1084/jem.20210446",

language = "English",

volume = "218",

journal = "Journal of Experimental Medicine",

issn = "0022-1007",

publisher = "Rockefeller University Press",

number = "6",

}

Orbak, Z, Aydemir, Ş, Bengi Celik, J, Kandemir, B, Aytekin, G, Kapakli, H, Yarar, V, Yosunkaya, A, Vatansev, H, Aytekin, C, Hancerli Torun, S, Nepesov, S, Coskuner, T, Sözeri, B, Kendir Demirkol, Y, Kasapcopur, O, Yıldız, M, Sevketoglu, E, Hatipoğlu, N, Özçelik, T, Yesilbas , O, Gayretli Aydin, ZG, Sediva, A, Klocperk, A, Bloomfield, M, Meyts, I, Delafontaine, S, Haerynck, F, Hoste, L, Shahrooei, M, Marque, L, Farela Neves, J, Novelli, G, Novelli, A, Aiuti, A, Casari, G, Aziz Bousfiha, A, Zaid Almuhsen, S, Sobh, A, Gagro, A, Bajolle, F, Bonnet, D, Lebon, P, Lei, W, Lee, D, Seeleuthner, Y, Zhang, P, Maglorius, M, Philippot, Q, Pelham, S, Bastard, P, Zhang, Q, Jouanguy, E, Puel, A, Herberg, J, Kuijpers, TW, Bellos, E, Kaforou, M, Menikou, S, Pan-Hammarström, Q, Hammarström, L, Abolhassani, H, Bryceson, Y, Condino-Neto, A, Prando, C, Yumi Bando, S, Cavalcanti, A, Fellay, J, Blanchard-Rohner, G, Mansouri, D, Mahmoudi, S, Boyarchuk, O, Volokha, A, Bondarenko, A, Stepanovskiy, Y, Mogensen, T, van de Beek, D, Andreakos, E, Papadaki, M, Abou Tayoun, A, Halwani, R, Al-Mulla, F, Franco, JL, Lau, Y-L, Kwan, M, Imai, K, Okada, S, Bolze, A, Butte, MJ, Hsieh, E, Drolet, BA, Arkin, L, Itan, Y, Maniatis, T, Arditi, M, Cooper, M, Schmitt, E, Chakravorty, S, Anderson, MS & Su, HC & Notarangelo, LD 2021, 'SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease?', Journal of Experimental Medicine, vol. 218, no. 6, e20210446. https://doi.org/10.1084/jem.20210446

TY - JOUR

T1 - SARS-CoV-2-related MIS-C

T2 - A key to the viral and genetic causes of Kawasaki disease?

AU - Sancho-Shimizu, Vanessa

AU - Brodin, Petter

AU - Cobat, Aurélie

AU - Biggs, Catherine M.

AU - Toubiana, Julie

AU - Lucas, Carrie L.

AU - Henrickson, Sarah E.

AU - Belot, Alexandre

AU - Tangye, Stuart G.

AU - Milner, Joshua D.

AU - Levin, Michael

AU - Abel, Laurent

AU - Bogunovic, Dusan

AU - Casanova, Jean Laurent

AU - Zhang, Shen Ying

AU - Haddad, Elie

AU - Beland, Kathie

AU - Pujol, Aurora

AU - Schlüter, Agatha

AU - Planas-Serra, Laura

AU - Aguilera-Albesa, Sergio

AU - Valencia-Ramos, Juan

AU - Rodríguez-Palmero, Agustí

AU - Gut, Marta

AU - Rivière, Jacques G.

AU - Colobran, Roger

AU - Soler-Palacin, Pere

AU - Rodriguez-Gallego, Carlos

AU - de Diego, Rebeca Perez

AU - Flores, Carlos

AU - Alsina, Laia

AU - Blazquez-Gamero, Daniel

AU - Jordan, Iolanda

AU - Keles, Sevgi

AU - Emiroglu, Melike

AU - Akcan, Ozge Metin

AU - Alkan, Gulsum

AU - Aytekin, Selma Erol

AU - Gul, Yahya

AU - Tüter Öz, Şadiye Kübra

AU - Bozdemir, Sefika Elmas

AU - Bayhan, Gulsum Iclal

AU - Yüksek, Saliha Kanık

AU - Parlakay, Aslınur Özkaya

AU - Gülhan, Belgin

AU - Yahşi, Aysun

AU - Kilic, Ahmet Osman

AU - Karbuz, Adem

AU - Erdeniz, Emine Hafize

AU - Özkan, Esra Akyüz

AU - Orbak, Zerrin

AU - Aydemir, Şehnaz

AU - Bengi Celik,, Jale

AU - Kandemir, Bahar

AU - Aytekin, Gökhan

AU - Kapakli, Hasan

AU - Yarar, Volkan

AU - Yosunkaya, Alper

AU - Vatansev, Hulya

AU - Aytekin, Caner

AU - Hancerli Torun, Selda

AU - Nepesov, Serdar

AU - Coskuner, Taner

AU - Sözeri, Betül

AU - Kendir Demirkol, Yasemin

AU - Kasapcopur, Ozgur

AU - Yıldız, Mehmet

AU - Sevketoglu, Esra

AU - Hatipoğlu, Nevin

AU - Özçelik, Tayfun

AU - Yesilbas , Osman

AU - Gayretli Aydin, Zeynep Gökçe

AU - Sediva, Anna

AU - Klocperk, Adam

AU - Bloomfield, Marketa

AU - Meyts, Isabelle

AU - Delafontaine, Selket

AU - Haerynck, Filomeen

AU - Hoste, Levi

AU - Shahrooei, Mohammad

AU - Marque, Laura

AU - Farela Neves, João

AU - Novelli, Giuseppe

AU - Novelli, Antonio

AU - Aiuti, Alessandro

AU - Casari, Giorgio

AU - Aziz Bousfiha, Amed

AU - Zaid Almuhsen, Saleh

AU - Sobh, Ali

AU - Gagro, Alenka

AU - Bajolle, Fanny

AU - Bonnet, Damien

AU - Lebon, Pierre

AU - Lei, Weite

AU - Lee, Danyel

AU - Seeleuthner, Yoann

AU - Zhang, Peng

AU - Maglorius, Majistor

AU - Philippot, Quentin

AU - Pelham, Simon

AU - Bastard, Paul

AU - Zhang, Qian

AU - Jouanguy, Emmanuelle

AU - Puel, Anne

AU - Herberg, Jethro

AU - Kuijpers, Taco W

AU - Bellos, Evangelos

AU - Kaforou, Myrsini

AU - Menikou, Stephanie

AU - Pan-Hammarström, Qiang

AU - Hammarström, Lennart

AU - Abolhassani, Hassan

AU - Bryceson, Yenan

AU - Condino-Neto, Antonio

AU - Prando, Carolina

AU - Yumi Bando, Silvia

AU - Cavalcanti, Andre

AU - Fellay, Jacques

AU - Blanchard-Rohner, Geraldine

AU - Mansouri, Davood

AU - Mahmoudi, Shima

AU - Boyarchuk, Oksana

AU - Volokha, Alla

AU - Bondarenko, Anastasiia

AU - Stepanovskiy, Yuriy

AU - Mogensen, Trine

AU - van de Beek, Diederik

AU - Andreakos, Evangelos

AU - Papadaki, Maria

AU - Abou Tayoun, Ahmad

AU - Halwani, Rabih

AU - Al-Mulla, Fahd

AU - Franco, José Luis

AU - Lau, Yu-Lung

AU - Kwan, Mike

AU - Imai, Kohsuke

AU - Okada, Satoshi

AU - Bolze, Alexandre

AU - Butte, Manish J.

AU - Hsieh, Elena

AU - Drolet, Beth A

AU - Arkin, Lisa

AU - Itan, Yuval

AU - Maniatis, Tom

AU - Arditi, Moshe

AU - Cooper, Megan

AU - Schmitt, Erica

AU - Chakravorty, Samya

AU - Anderson, Mark S.

AU - Su, Helen C.

AU - Notarangelo, Luigi D.

PY - 2021/4/27

Y1 - 2021/4/27

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85105542114&partnerID=8YFLogxK

U2 - 10.1084/jem.20210446

DO - 10.1084/jem.20210446

M3 - Review article

C2 - 33904890

AN - SCOPUS:85105542114

SN - 0022-1007

VL - 218

JO - Journal of Experimental Medicine

JF - Journal of Experimental Medicine

IS - 6

M1 - e20210446

ER -

SARS-CoV-2-related MIS-C: A key to the viral and genetic causes of Kawasaki disease?

Abstract

Access to Document

Other files and links

Fingerprint

Cite this