Rheumatology International

, Volume 39, Issue 10, pp 1829–1838 | Cite as

Kawasaki disease and immunodeficiencies in children: case reports and literature review

  • Francisco Rivas-Larrauri
  • Lorena Aguilar-Zanela
  • Paola Castro-Oteo
  • Luis Adrian Rosales-Hernandez
  • Francisco Otero-Mendoza
  • Gabriela López-Herrera
  • Javier Ordoñez-Ortega
  • Martín Garrido-García
  • Marco Antonio Yamazaki-NakashimadaEmail author
Case Based Review


Kawasaki disease (KD) has features that appear supporting an infectious cause with a secondary deranged inflammatory/autoimmune response. The association of KD in adults with human immunodeficiency virus infection and the presence of KD in patients with immunodeficiency disorders support the infectious theory. We present four KD patients associated with immunodeficiencies: one with X-linked agammaglobulinemia, one with HIV infection, and two with leukemia; one of these patients also had Down syndrome. We did a literature search to find out all reported cases of immunodeficiency with KD in children. In immunodeficiency disorders, the inability of the immune system to eradicate the pathogens coupled to an exaggerated inflammatory response, especially in chronic granulomatous disease, may lead to the development of KD. The study of patients with immunodeficiencies complicated with KD may shed light into the etiopathogenesis of the disease.


Kawasaki disease Intravenous immunoglobulins Immunodeficiency Primary immunodeficiency disorders HIV Malignancy 



The authors thank Dr. Alberto Unzueta for editing and helpful discussions.

Author contributions

Case 1 was diagnosed and followed up by LAZ, PCO, LARH, JOO and MAYN. FRL, MGG, and MAYN were involved in the caring of Case 2 and Case 3, and Case 4 was diagnosed and followed up by FOM, GLH, and MAYN. LAZ, PCO, LARH, and MAYN wrote the manuscript. Literature data were searched and analyzed by all the authors. The final version was read corrected and approved by all the authors.


The authors received no financial support for the research, authorship, and/or publication of this article.

Compliance with ethical standards

Conflict of interest

Dr. Marco Antonio Yamazaki-Nakashimada has received lecture fees from Shire, CSL Behring and Octapharma, and the other authors declare that they do not have any conflict of interests.

Ethical approval

All the procedures performed in this study were in accordance with the ethical standards of the institutional and or national research committee and with the 1964 Helsinki Declaration and its later amendments.

Informed consent

Written informed consent was obtained from the patients for publication of the case reports.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Clinical Immunology DepartmentInstituto Nacional de PediatríaMexico CityMexico
  2. 2.Pediatric Infectology DepartmentInstituto Nacional de PediatríaMexico CityMexico
  3. 3.Immunodeficiencies Research UnitInstituto Nacional de PediatríaMexico CityMexico
  4. 4.Pediatric Cardiology DepartmentInstituto Nacional de PediatríaMexico CityMexico

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