Journal article
Pediatric rheumatologists' perspectives on diagnosis, treatment, and outcomes of Sjogren disease in children and adolescents
Pediatric rheumatology online journal, Vol.20(1), pp.1-79
09/05/2022
DOI: 10.1186/s12969-022-00740-4
PMCID: PMC9446526
PMID: 36064423
Abstract
Background Sjogren disease in children and adolescents (pedSD) presents differently than adult disease. Diagnosis and classification are controversial, optimal treatment is unknown and outcomes are poorly understood. Here, we describe the current perspectives of pediatric rheumatologists on diagnosis, treatment, and outcomes of pedSD. Methods A voluntary, 17-question survey was distributed to providers in the Childhood Arthritis and Rheumatology Research Alliance and/or the American College of Rheumatology Childhood Sjogren's Study Group at the 2020 Convergence Virtual Conference. Findings are reported using descriptive statistics and chi-square testing. Results Of 465 eligible providers, 157 (34%) responded with 135 (29%) completing the survey. The majority (85%) saw five or fewer patients with pedSD in the past year. Parotitis, dry eye and/or dry mouth, and constitutional symptoms were among the most specific and common clinical features. Most providers (77%) used clinical judgment guided by adult criteria for diagnosis. The vast majority (86-99%) of survey participants indicated routine use of serologic testing, while salivary gland ultrasound, minor salivary gland biopsy and other diagnostic tests were less often used. The most commonly prescribed systemic immunomodulators were hydroxychloroquine, corticosteroids, methotrexate, rituximab, and mycophenolate. Seven providers reported malignancy in a patient with pedSD, including one death. Conclusions Pediatric rheumatologists diagnose and treat pedSD; however, most only see a few patients per year and rely on clinical judgment and laboratory testing for diagnosis. Treatment frequently includes systemic immunomodulators and malignancies are reported. More studies are needed to better understand natural history, risk factors, and the impact of interventions on outcomes.
Details
- Title: Subtitle
- Pediatric rheumatologists' perspectives on diagnosis, treatment, and outcomes of Sjogren disease in children and adolescents
- Creators
- Rachel L. Randell - Duke UniversitySara M. Stern - University of UtahHeather Van Mater - Duke UniversityLaura E. Schanberg - Duke UniversityScott M. Lieberman - Univ Iowa, Carver Coll Med, Stead Family Dept Pediat, Div Rheumatol Allergy & Immunol, Iowa City, IA USAMatthew L. Basiaga - Mayo ClinicCARRA Investigators
- Resource Type
- Journal article
- Publication Details
- Pediatric rheumatology online journal, Vol.20(1), pp.1-79
- DOI
- 10.1186/s12969-022-00740-4
- PMID
- 36064423
- PMCID
- PMC9446526
- NLM abbreviation
- Pediatr Rheumatol Online J
- ISSN
- 1546-0096
- eISSN
- 1546-0096
- Publisher
- Springer Nature
- Number of pages
- 7
- Grant note
- U19AR069522 / NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) PaCr-2017C2-8177 / Patient-Centered Outcomes Research Institute; Patient-Centered Outcomes Research Institute - PCORI R01EY027731 / National Eye Institute (NEI) of the NIH National Institute of General Medical Sciences; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of General Medical Sciences (NIGMS) Sjogren's Foundation T32GM086330 / Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD) of the National Institutes of Health (NIH) Childhood Arthritis and Rheumatology Research Alliance (CARRA)
- Language
- English
- Date published
- 09/05/2022
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984353945302771
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