BCGitis as the primary manifestation of chronic granulomatous disease

Nastaran Khalili; Iraj Mohammadzadeh; Neda Khalili; Raúl Jimenez Heredia; Samaneh Zoghi; Kaan Boztug; Nima Rezaei

doi:10.1016/j.idcr.2020.e01038

BCGitis as the primary manifestation of chronic granulomatous disease

IDCases. 2020 Dec 29:23:e01038. doi: 10.1016/j.idcr.2020.e01038. eCollection 2021.

Authors

Nastaran Khalili^{1

2}, Iraj Mohammadzadeh³, Neda Khalili^{1

2}, Raúl Jimenez Heredia⁴, Samaneh Zoghi^{4

5

6}, Kaan Boztug^{4

5

7}, Nima Rezaei^{8

9

10}

Affiliations

¹ Cancer Immunology Project (CIP), Universal Scientific Education and Research Network (USERN), Tehran, Iran.
² School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
³ Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran.
⁴ Ludwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
⁵ CeMM Research Centre for Molecular Medicine of the Austrian Academy of Sciences, Vienna, Austria.
⁶ Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Vienna, Austria.
⁷ St Anna Children's Hospital, Department of Paediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria.
⁸ Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
⁹ Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran.
¹⁰ Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran.

Abstract

Patients with primary immunodeficiency disease (PID) are not only vulnerable to mycobacterial disease, but are also more likely to develop adverse events following BCG vaccination. These events can range from regional disease (BCGitis) to disseminated disease (BCGosis). Chronic granulomatous disease (CGD), which is characterized by impaired leukocyte phagocytic function, is one of the many inherited PIDs that increase the body's susceptibility to recurrent bacterial and fungal infections. Here, we report a 6-year-old boy with no significant past medical history who presented with progressive lymphadenopathy six years after BCG vaccination. He was later diagnosed with CGD on further evaluation.

Keywords: BCG vaccination; BCGiosis; BCGitis; Chronic granulomatous disease; Lymphadenopathy; Primary immunodeficiency disease.

Publication types

Case Reports