Journal article
Childhood Sjögren syndrome: insights from adults and animal models
Current opinion in rheumatology, Vol.25(5), pp.651-657
09/2013
DOI: 10.1097/BOR.0b013e328363ed23
PMCID: PMC4049091
PMID: 23917159
Abstract
Sjögren syndrome is a chronic autoimmune disease affecting lacrimal and salivary glands that often is accompanied by extraglandular disease manifestations. Although common in adults, the prevalence and prognosis of childhood Sjögren syndrome are unknown, in part due to lack of child-specific diagnostic and classification criteria. This review discusses difficulties in diagnosing childhood Sjögren syndrome and highlights recent findings in Sjögren syndrome treatment and pathogenesis from studies in adults and animal models over the past 18 months.
Studies of rituximab show some therapeutic potential in adult Sjögren syndrome, whereas newer modalities including gene therapy and mesenchymal stem cell transfer are promising. The pathogenesis of Sjögren syndrome is emerging, including roles of T and B lymphocytes, autoantibodies, interferons, and glandular epithelial cells. Specific recent notable findings in Sjögren syndrome pathogenesis include identification of a type II interferon signature in salivary glands of Sjögren syndrome patients, characterization of salivary gland-infiltrating T-cell subsets, and characterization of antimuscarinic acetylcholine receptor type 3 autoantibodies.
Childhood Sjögren syndrome is a poorly defined and underdiagnosed autoimmune disease that requires child-specific criteria in order to study disease burden and prognosis. Studies in adults and animal models continue to elucidate new potential diagnostic and therapeutic targets, which may be relevant for childhood Sjögren syndrome.
http://links.lww.com/COR/A3.
Details
- Title: Subtitle
- Childhood Sjögren syndrome: insights from adults and animal models
- Creators
- Scott M Lieberman - Division of Rheumatology, The Children's Hospital of Philadelphia and Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA. scott-lieberman@uiowa.edu
- Resource Type
- Journal article
- Publication Details
- Current opinion in rheumatology, Vol.25(5), pp.651-657
- DOI
- 10.1097/BOR.0b013e328363ed23
- PMID
- 23917159
- PMCID
- PMC4049091
- NLM abbreviation
- Curr Opin Rheumatol
- ISSN
- 1040-8711
- eISSN
- 1531-6963
- Grant note
- K08 EY022344 / NEI NIH HHS K08-EY022344 / NEI NIH HHS
- Language
- English
- Date published
- 09/2013
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984093372402771
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