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Pediatric IgG4-related disease: a descriptive review
Satoshi Hara1, Misaki Yoshida2, Hajime Sanada1
1Department of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
Expert Review of Clinical Immunology
|October 24, 2023
Summary
Pediatric IgG4-related disease (IgG4-RD) presents differently than adult forms, with more surface organ involvement and no male predominance. Early recognition is crucial as few cases meet diagnostic criteria.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Systemic Inflammatory Diseases
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a rare fibroinflammatory condition.
- It typically affects elderly males but can occur in children.
- Delayed diagnosis in pediatric cases can lead to poor outcomes.
Purpose of the Study:
- To review and characterize pediatric IgG4-related disease.
- To compare pediatric IgG4-RD with adult cases.
- To assess diagnostic criteria adherence in published pediatric cases.
Main Methods:
- Descriptive review of published literature (case reports, cohort studies, reviews) on PubMed.
- Analysis of pediatric IgG4-RD cases including demographics, organ involvement, serology, histology, and treatment.
- Evaluation of case adherence to current IgG4-RD classification and diagnostic criteria.
Main Results:
- Pediatric IgG4-RD shows distinct characteristics from adult forms, notably in sex distribution and organ involvement.
- No clear male dominance observed in pediatric cases.
- Ophthalmic and other surface organ involvement is more common in children, often presenting indolent and unilaterally.
- Only approximately 20% of reviewed pediatric cases met established IgG4-RD diagnostic criteria.
Conclusions:
- Pediatric IgG4-RD differs significantly from adult IgG4-RD.
- Diagnosis in children is challenging due to atypical presentations and indolent nature.
- Increased physician awareness and exclusion of mimickers are vital.
- International collaboration is needed to improve evidence for diagnosis and treatment.
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