Pediatric primary Sjögren syndrome presenting with bilateral ranulas

A case report and systematic review of the literature

Casey Means, Mark A. Aldape, Ericka King

Research output: Contribution to journalReview article

4 Citations (Scopus)

Abstract

Context Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. Objective This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children. Data sources Ovid MEDLINE. Study selection A MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Data extraction Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Results Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. Limitations All studies were retrospective chart reviews, case series or case reports. Conclusion This is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome.

Original languageEnglish (US)
Pages (from-to)11-19
Number of pages9
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume101
DOIs
StatePublished - Oct 1 2017

Fingerprint

Ranula
Pediatrics
MEDLINE
Parotitis
Mouth Floor
Information Storage and Retrieval
Salivary Glands
Mouth
Meta-Analysis
Retrospective Studies

Keywords

  • Child
  • Keratoconjunctivitis
  • Parotitis
  • Primary Sjögren syndrome
  • Ranula
  • Sicca
  • Xerostomia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology

Cite this

Pediatric primary Sjögren syndrome presenting with bilateral ranulas : A case report and systematic review of the literature. / Means, Casey; Aldape, Mark A.; King, Ericka.

In: International Journal of Pediatric Otorhinolaryngology, Vol. 101, 01.10.2017, p. 11-19.

Research output: Contribution to journalReview article

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title = "Pediatric primary Sj{\"o}gren syndrome presenting with bilateral ranulas: A case report and systematic review of the literature",
abstract = "Context Primary Sj{\"o}gren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sj{\"o}gren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sj{\"o}gren syndrome. Objective This study aims to describe a novel presentation of juvenile primary Sj{\"o}gren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sj{\"o}gren syndrome in children. Data sources Ovid MEDLINE. Study selection A MEDLINE literature search was performed using the following search terms: primary, Sj{\"o}gren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Data extraction Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Results Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sj{\"o}gren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sj{\"o}gren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. Limitations All studies were retrospective chart reviews, case series or case reports. Conclusion This is the first report of a child presenting with floor of mouth ranulas in association with Sj{\"o}gren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sj{\"o}gren syndrome.",
keywords = "Child, Keratoconjunctivitis, Parotitis, Primary Sj{\"o}gren syndrome, Ranula, Sicca, Xerostomia",
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N2 - Context Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. Objective This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children. Data sources Ovid MEDLINE. Study selection A MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Data extraction Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Results Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. Limitations All studies were retrospective chart reviews, case series or case reports. Conclusion This is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome.

AB - Context Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. Objective This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children. Data sources Ovid MEDLINE. Study selection A MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Data extraction Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Results Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. Limitations All studies were retrospective chart reviews, case series or case reports. Conclusion This is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome.

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KW - Sicca

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