Unique Aspects of Pediatric Sjögren Disease

Randell, Rachel L.

doi:10.1016/j.rdc.2021.07.008

Cited by 8 publications

(9 citation statements)

References 59 publications

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“…As a second parameter, we evaluated glandular vascularization, using a consensus-based color Doppler semiquantitative score as suggested by Hočevar et al [12] Finally, histopathological parameters were assessed through LSG biopsy, according to Chisholm and Mason scoring system, which includes 5 grades from 0 to 4, based on the presence of slight or moderate lymphocytic infiltration and/or focus of lymphocytes [13,14]). We considered as a positive biopsy any grade of focal sialadenitis, that is, a focus score > 0 foci/4 mm 2 [3,4,15].…”

Section: Ultrasonography and Biopsymentioning

confidence: 99%

Ultra-High-Frequency Ultrasonography of Labial Glands in Pediatric Sjögren’s Disease: A Preliminary Study

Marrani,

Fulvio,

Virgili

et al. 2023

Diagnostics

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Sjögren’s disease (SD) is a chronic autoimmune disease primarily affecting lacrimal and salivary glands. The diagnosis of pediatric SD mostly relies on clinical suspect, resulting in a significant diagnostic delay. Recently, ultrahigh-frequency ultrasound (UHFUS) of labial glands has been proposed as a diagnostic method in adults with suspected SD. Until now, there have been no studies about UHFUS in pediatric diagnostic work-up. The aim of the study was to evaluate the potential role of UHFUS of minor salivary glands in pediatric SD. Consecutive pediatric patients with a diagnosis of pediatric SD seen at AOU Meyer IRCSS were evaluated. Intraoral UHFUS scan of the lip mucosa was performed with Vevo MD equipment, using a 70 MHz probe with a standardized protocol and the images were independently reviewed by two operators. Lip salivary glands were assessed by using a four-grade semiquantitative scoring system for parenchymal alteration and vascularization. Twelve patients were included. When applying UHFUS to this cohort of patients, all patients showed a UHFUS grade of ≥1 with 8/12 showing a mild glandular alteration (i.e., grade 1), 2/12 a moderate glandular alteration (i.e., grade 2) and finally 2/12 a severe glandular alteration (i.e., grade 3). Moderate intraglandular vascularization was seen in 9/12, with only 3/12 showing mild intraglandular vascularization. Due to limited size of the sample, the relationship between histological findings, autoantibodies status and UHFUS grade could not be performed. This preliminary study seems to report UHFUS as feasibility technique to identify salivary gland alterations in children with a clinical suspect of SD.

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Section: Ultrasonography and Biopsymentioning

confidence: 99%

Ultra-High-Frequency Ultrasonography of Labial Glands in Pediatric Sjögren’s Disease: A Preliminary Study

Marrani,

Fulvio,

Virgili

et al. 2023

Diagnostics

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“…Childhood-onset or juvenile SjD (jSjD) is defined as a disease manifesting before 18 years of age [8,9], with a Extended author information available on the last page of the article peak age of 10-14 years [5]. No established and validated classification criteria are available for jSjD [9,10], so that general practice is to use the American-European Consensus Group (AECG) criteria [11] and the American College of Rheumatology/European Alliance of Associations for Rheumatology (EULAR) criteria [12] for adult SjD.…”

Section: Introductionmentioning

confidence: 99%

“…Extra-glandular manifestations include interstitial lung disease, renal disease (e.g. renal tubular acidosis), vasculitis, central nervous system (CNS) involvement, leukopenia, thrombocytopenia, anaemia, lymphadenopathy and musculoskeletal manifestations (arthralgia, arthritis, myalgia) [3,5,6,8,9]. Lymphoma is a rare complication, but still, it is reported in jSjD [17,18].…”

Section: Introductionmentioning

confidence: 99%

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Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

Lercara,

Malattia,

Hysa

et al. 2024

Clin Rheumatol

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Introduction Juvenile Sjögren’s disease (jSjD) is a rare autoimmune disease characterized by exocrine gland involvement and systemic manifestations, including small vessel vasculitis and Raynaud’s phenomenon (RP). We aimed to investigate the microvascular status in jSjD patients by nailfold videocapillaroscopy (NVC) and the potential correlations with clinical and serological features. Methods Clinical data from thirteen consecutive jSjD patients (11 females and 2 males), with a mean age of 16 ± 4 years, diagnosed before 16 years of age (mean age at diagnosis 12 ± 3) according to the 2016 American College of Rheumatology/EULAR criteria for adult SjD, were collected including age- and sex-matched healthy controls (HCs). Clinical, laboratory, and instrumental data were collected, together with NVC examination. Non-specific and specific NVC parameters were investigated, such as capillary density, capillary dilations, giant capillaries, microhaemorrhages and abnormal shapes. Associations between NVC findings and clinical/serological features were explored and analysed using parametrical and non-parametrical tests. Results Capillary density reduction correlated significantly with articular involvement (arthralgias) (p = 0.024). Microhaemorrhages correlated with lower C3 levels (p = 0.034). No specific NVC pattern for jSjD was identified, whereas abnormal capillary shapes were significantly higher in jSjD patients than HCs (p = 0.005). NVC abnormalities were not associated with SjD-specific instrumental tests (biopsy, imaging, Schirmer’s test). RP was present in 8% of jSjD patients. Conclusions The reduction of capillary density, as well as microhaemorrhages at NVC analysis, are significantly associated with some clinical aspects like articular involvement and serum biomarkers (C3 reduction). The NVC is suggested as safe and further analysis in jSjD patients. Key Points• Juvenile Sjögren’s disease (jSjD) exhibits a higher rate of abnormal shapes at nailfold videocapillaroscopy (NVC) compared to matched healthy controls.• Articular involvement in jSjD is significantly associated with a lower capillary number at NVC.• Microhaemorrhages on NVC are associated with lower C3 levels, prompting this finding as a putative novel negative biomarker/prognostic factor for jSjD.

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“…While uncommon in childhood, SD is increasingly recognized in patients less than 18 years of age (pedSD) [1][2][3]. The clinical presentation of pedSD often differs from adult disease, with a lower frequency of classic "sicca symptoms" (xerostomia and xerophthalmia) and a higher frequency of glandular swelling such as parotitis [4]. Additionally, many diagnostic tests used to define SD in adults [5] lack feasibility and/or age-adjusted normative values in children.…”

Section: Introductionmentioning

confidence: 99%

Pediatric rheumatologists’ perspectives on diagnosis, treatment, and outcomes of Sjögren disease in children and adolescents

Randell

Stern²,

Mater³

et al. 2022

Pediatr Rheumatol

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Background Sjögren 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 Sjögren’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.

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Unique Aspects of Pediatric Sjögren Disease

Cited by 8 publications

References 59 publications

Ultra-High-Frequency Ultrasonography of Labial Glands in Pediatric Sjögren’s Disease: A Preliminary Study

Ultra-High-Frequency Ultrasonography of Labial Glands in Pediatric Sjögren’s Disease: A Preliminary Study

Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

Pediatric rheumatologists’ perspectives on diagnosis, treatment, and outcomes of Sjögren disease in children and adolescents

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