Pediatric IgG4-related disease: a descriptive review

ABSTRACT

Introduction

IgG4-related disease (IgG4-RD) is an immune-mediated systemic fibroinflammatory condition characterized by serum IgG4 elevation and IgG4-positive plasma cell infiltration into various organs. It generally occurs in elderly males. Pediatric cases have been reported, albeit rarely, accordingly lack of recognition of such cases could delay therapeutic intervention leading to poorer outcomes.

Areas covered

The present review is a descriptive review of all published case reports, cohort studies, and reviews of pediatric IgG4-RD listed in PubMed. Characteristics of pediatric IgG4-RD were clarified, including sex, organ involvement, serological and histological findings, and treatment. We assessed how many published cases met current classification and comprehensive diagnostic criteria.

Expert opinion

The characteristics of pediatricIgG4-RD differed from adult IgG4-RD in terms of sex and involved organs. There was no clear male dominance in numbers of cases, and surface organ involvement such as ophthalmic diseases were more common in the pediatric IgG4-RD. Organ involvement tended to be indolent and unilateral, causing difficulty in definitively diagnosing pediatric IgG4-RD. Only about 20% of published cases met IgG4-RD classification or comprehensive diagnostic criteria. Physicians should be careful in diagnosing pediatric IgG4-RD after excluding mimickers. International collaboration toward high-quality evidence to support diagnosis and treatment of pediatric IgG4-RD is advised.

KEYWORDS:

• Classification criteria
• comprehensive diagnostic criteria
• IgG4-related disease
• international collaboration
• literature review
• ophthalmic disease
• pediatric
• sex

Article highlights

• The characteristics of IgG4-RD in children markedly differ from those in adults by sex and organ involvement: There is no clear male dominance in pediatric IgG4-RD. Surface organ involvement such as ophthalmic diseases are more common in pediatric than adult IgG4-RD cases. Important to note- organ involvement tends to be indolent and unilateral in children.

• Only about 20% of published pediatric cases met IgG4-RD classification criteria or comprehensive diagnostic criteria. Physicians must make sure to exclude mimickers and differential diagnoses such as systemic vasculitis and malignancy to definitively diagnose pediatric IgG4-RD.

• International collaboration is essential to amass high-quality evidence to support diagnosis and treatment of pediatric IgG4-RD.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Acknowledgments

A representative case of pediatric IgG4-RD was presented as Abstract at the 14th Scientific Meeting of Japanese Society of IgG4-RD on March 4^th, 2023. We thank David Price for his critical proofreading of our manuscript.

Additional information

Funding

This paper was not funded.