Book chapter
33 - Sjögren Disease and Immunoglobulin-G4–Related Disease
Textbook of Pediatric Rheumatology, pp.489-499
Elsevier, Ninth Edition
2026
DOI: 10.1016/B978-0-443-11980-4.00033-7
Abstract
Sjögren disease is a heterogeneous chronic systemic autoimmune rheumatic disease, with manifestations ranging from localized glandular disease to systemic and extra-glandular organ system involvement. This disease has been considered to be rare in children and adolescents; however, it has almost certainly been underdiagnosed and is more common than previously realized. This chapter reviews the epidemiology of this condition in childhood, the spectrum of clinical manifestations including those reported in recent large series, the pathophysiology, diagnostic and classification criteria, and the newest treatment recommendations.
IgG4-related diseases are a relatively new group of inflammatory diseases characterized by very high serum levels of IgG4, a characteristic “storiform” fibrosis, obliterative phlebitis, and mild to moderate eosinophilia in affected tissues. This chapter reviews the clinical manifestations and treatment of this condition in children.
Details
- Title: Subtitle
- 33 - Sjögren Disease and Immunoglobulin-G4–Related Disease
- Creators
- Scott M. LiebermanCoziana Ciurtin
- Resource Type
- Book chapter
- Publication Details
- Textbook of Pediatric Rheumatology, pp.489-499
- Edition
- Ninth Edition
- DOI
- 10.1016/B978-0-443-11980-4.00033-7
- Publisher
- Elsevier; Philadelphia, PA
- Language
- English
- Date published
- 2026
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9985166870302771
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