Childhood Sjogren's syndrome: An Italian case series and a literature review-based cohort

Marino, Achille; Romano, Micol; Giani, Teresa; Gaggiano, Carla; Costi, Stefania; Singh, Revika; Mehta, Jay; Cimaz, Rolando

doi:10.1016/j.semarthrit.2020.11.004

Cited by 16 publications

(13 citation statements)

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“…Combined with cases in our hospital and reported literature, we present some reasons why existing diagnostic criteria may be inadequate for children. First, several recent large or multicenter cohort studies found that among C-pSS patients, parotitis was the most common symptom at presentation, although dry mouth and dry eyes were commonly reported, and systemic symptoms, including unexplained fever, arthralgia, dental caries, and organ involvement were more likely to happen (8)(9)(10)(11)(12). Consistently, the most common manifestation of pSS in our study was rash (51%), especially purpura, followed by parotitis (36.7%), whereas hematology was the most common organ (65.3%).…”

Section: Discussionmentioning

confidence: 99%

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Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

et al. 2023

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IntroductionThe initial presentations of childhood-onset primary Sjögren’s syndrome (C-pSS) vary, making diagnosis challenging. We aimed to improve the diagnosis and evaluation of C-pSS by summarizing its clinical and laboratory features.MethodsA total of 49 patients with C-pSS between July 2015 and August 2022 in the Department of Rheumatology and Immunology of Shanghai Children's Medical Centre were enrolled in this study. Their clinical manifestations and laboratory examinations of these patients were compared based on the presence or absence of thrombocytopenia and parotitis and whether the immunological markers, including anti-nuclear antibodies (ANA), rheumatoid factor (RF), anti-Ro52/SSA antibodies (anti-SSA/Ro52), anti-Ro60/SSA antibodies (anti-SSA/Ro60), and anti-Ro/SSB antibodies (anti-SSB), were positive.ResultsThe mean age at C-pSS diagnosis was 10.34 ± 3.45 years, and the ratio of boys to girls was 1:6. In the thrombocytopenia group, parotitis (P = 0.044), organ involvement except for hematology (P = 0.002), positive anti-SSB (P = 0.004), and positive RF (P = 0.001) were less frequently observed. Complement C4 (P = 0.038) and white blood cells (P = 0.002) levels decreased and increased significantly, respectively. Anti-SSB (P = 0.010) and RF (P = 0.004) positivity were independent potential protective factors against thrombocytopenia in patients with C-pSS. In the parotitis group, higher ANA titers (P = 0.027), higher focus scores on labial gland biopsy (P = 0.024), and positive RF (P = 0.001), anti-SSA/Ro60 (P = 0.003), and anti-SSB (P = 0.001) were observed more frequently. Furthermore, positive anti-SSB (P = 0.012) and positive RF (P = 0.028) were independent risk factors for parotitis in patients with C-pSS. The hemoglobin level was significantly lower in patients with positive anti-SSA/Ro52 and positive anti-SSA/Ro60 results (P = 0.022 and P = 0.029, respectively), while immunoglobulin G level was significantly higher in patients in the same group (P = 0.048 and P = 0.007, respectively).ConclusionsPositive anti-SSB and positive RF values may be independent potential protective factors of thrombocytopenia in patients with C-pSS. In contrast, positive anti-SSB and positive RF were independent risk factors of parotitis in patients with C-pSS. More studies are needed to reveal the diagnostic role and pathogenic mechanism of immunological markers in C-pSS.

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Section: Discussionmentioning

confidence: 99%

“…It was found that C-pSS involves approximately 1% of patients with pSS, with a male-to-female ratio of approximately 1:5-12 and a peak age of 10-14 years. Meanwhile, clinical phenotypes are dominated by parotid enlargement and systemic symptoms (7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

et al. 2023

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“…This is characterized by recurrent parotid gland inflammation (about 67 % of patients) and arthralgia. Laboratory findings (rheumatic factor, ANA, Ro/SS‐A antibodies) may be normal in patients with juvenile SjS [24, 25].…”

Section: Epidemiologymentioning

confidence: 99%

Sjögren’s syndrome

André

Böckle

2022

J Deutsche Derma Gesell

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Sjögren's syndrome (SjS) is an autoimmune disease characterized by the triad of sicca symptoms, fatigue and pain. This diagnosis is usually made in women at the average age of 60 years. Diagnosis is made when sicca symptoms persist for more than three months, after the exclusion of possible differential diagnoses, and using the ACR/ EULAR 2016 classification criteria for SjS. Many organs can be affected in the course of this disease. Xerosis cutis and pruritus are the most common skin manifestations, followed by leukocytoclastic vasculitis and subacute cutaneous lupus erythematosus. In addition, SjS patients often have myoarthralgia and neuropsychiatric symptoms. In the long term, attention must be paid to the increased risk of cardiovascular disease and lymphoma. Due to the multiorgan involvement in SjS patients, interdisciplinary care is required.

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“…Dieses ist durch wiederholt auftretende Ohrspeicheldrüsenentzündungen (circa 67 % der Patienten) und Arthralgien gekennzeichnet. Die Laborbefunde (Rheumafaktor, ANA, Ro/SS‐A Antikörper) können beim juvenilen SjS unauffällig bleiben [24, 25].…”

Section: Epidemiologieunclassified

Sjögren‐Syndrom

André

Böckle

2022

J Deutsche Derma Gesell

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Das Sjögren-Syndrom (SjS) ist eine Autoimmunerkrankung aus dem Formenkreis der Kollagenosen, die durch die Trias Sicca-Symptomatik, Fatigue und Schmerz charakterisiert ist. Die Diagnose wird meist bei Frauen und oft erst spät, im Alter von durchschnittlich 60 Jahren, gestellt. Bei mehr als drei Monate andauernder Sicca-Symptomatik, die durch andere Differenzialdiagnosen nicht erklärbar ist, können die ACR/ EULAR 2016 Klassifikationskriterien für das SjS angewendet werden, um die Diagnose zu stellen. Im Rahmen des SjS können viele Organe betroffen sein. Die Xerosis cutis und der Pruritus sind die häufigsten Hautmanifestationen. Oftmals werden eine leukozytoklastische Vaskulitis und ein subakut kutaner Lupus erythematodes bei SjS-Patienten diagnostiziert. Zusätzlich haben SjS-Patienten häufig Myoarthralgien sowie neuropsychiatrische Symptome. Langfristig muss auf das erhöhte Risiko für kardiovaskuläre Erkrankungen sowie Lymphome geachtet werden. Aufgrund der Beteiligung vieler Organe bei SjS-Patienten ist ein interdisziplinärer Behandlungsansatz

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Childhood Sjogren's syndrome: An Italian case series and a literature review-based cohort

Cited by 16 publications

References 81 publications

Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

Clinical and laboratory features of childhood-onset primary Sjögren's syndrome: A retrospective study from China

Sjögren’s syndrome

Sjögren‐Syndrom

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