Interface between inborn errors of immunity and rheumatological disorders in children: A pediatrician’s conundrum

doi:10.5409/wjcp.118174

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World Journal of Clinical Pediatrics

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2219-2808

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.

World J Clin Pediatr. Sep 9, 2026; 15(3): 118174
Published online Sep 9, 2026. doi: 10.5409/wjcp.118174

Interface between inborn errors of immunity and rheumatological disorders in children: A pediatrician’s conundrum

Abarna Thangaraj, Ridhima Aggarwal, Soumyadeep Sarkar, Rakesh Kumar Pilania

Abarna Thangaraj, Ridhima Aggarwal, Soumyadeep Sarkar, Rakesh Kumar Pilania, Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India

Co-first authors: Abarna Thangaraj and Ridhima Aggarwal.

Author contributions: Thangaraj A, Aggarwal R, and Sarkar S conducted the literature review, performed the analysis, created the artwork, interpreted the data, and drafted the original manuscript; Pilania RK conceptualized and designed the study, and critically revised the manuscript. All authors prepared the draft and approved the submitted version. Thangaraj A and Aggarwal R contributed equally to this work and are co-first authors.

Conflict-of-interest statement: The authors declare no conflicts of interest.

Corresponding author: Rakesh Kumar Pilania, MD, DM, MAMS, Assoc-FAMS, Assoc-FNA, Associate Professor, Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India. kumarpilania007@gmail.com

Received: December 29, 2025
Revised: February 20, 2026
Accepted: April 3, 2026
Published online: September 9, 2026
Processing time: 217 Days and 15.2 Hours

Core Tip

Core Tip: Rheumatological disorders in children may represent the initial manifestation of inborn errors of immunity (IEIs), a rapidly expanding group of monogenic immune dysregulation syndromes. Increasing overlap exists between IEIs and pediatric rheumatologic diseases, including arthritis, vasculitis, cytopenias, lupus, macrophage activation syndrome, and polyautoimmunity. Early age of onset, atypical or severe disease, recurrent infections, multi-organ involvement, and poor response to standard therapy are key red flags. Early recognition and genetic diagnosis enable precision-based targeted therapies, significantly improving outcomes.