Improvement of Recurrent Parotitis by Sialendoscopy in Juvenile Sjogren’s Syndrome, Measured by Ultrasonography: Case Report with Literature Review

Submitted by:

Kristin Drew
University of Florida College of Medicine

Presenter(s):

Kristin Drew

Abstract

Sjogren syndrome (SS) is an autoimmune inflammatory condition which often affects female patients between 45 and 55 years of age and presents with sicca symptoms.1 However, SS can rarely occur in pediatric populations, namely Juvenile SS (JSS), which often presents recurrent parotitis, positive serology, neurological and renal manifestations.2 Salivary gland ultrasonography (SGUS) with a simplified score assessment of parenchyma dyshomogeneity is a highly specific test for primary SS.3 In this case report, we present the case of a 12 year-old female who was diagnosed with JSS based on the 2016 ACR/EULAR SS diagnostic criteria for adults and monitored for progress via SGUS throughout treatment involving sialendoscopy.

The patient with a history of type I diabetes mellitus presented with recurrent, tender swelling of her parotid glands refractory to antibiotic treatment, xerostomia, and keratoconjunctivitis sicca. Her lab work was negative for SSA and SSB antibodies but showed early markers for JSS with positive salivary protein 1 IgA autoantibody. A labial biopsy was then performed to reveal lymphocytic sialadenitis. On the basis of clinical symptoms, serologic markers, and positive lip biopsy, she was diagnosed with JSS. Pharmacologic treatment included 200mg Plaqenil to manage symptoms. She underwent bilateral parotid duct and gland sialendoscopy with 100mg Kenalog irrigation to each gland. Her SGUS score before sialendoscopy was 16, graded based on the Hocevar’s scoring system, with ultrasound showing multiple hypoechoic lesions.4 In the months following her sialendoscopy, her score reduced to the 10-11 range with resultant homogeneous findings on SGUS, indicating significant improvement of glandular inflammation. Eleven months post-sialendoscopy, she had a recurrent swelling of the right gland which resulted in increasing daily Plaqenil dose from 200mg to 300mg. Twelve months after her sialendoscopy, her SGUS score remained at 11.

Diagnosis of JSS is challenging and must draw from multiple elements including clinical presentation, serology for autoantibodies or cytopenia, and imaging. Since patients’ clinical phenotype can be heterogeneous, patients with negative SSA/SSB are still recommended to undergo a labial biopsy to rule out JSS.5 According to Theander and Mandl, SGUS is an effective method for diagnostic and prognostic purposes in diagnosing SS, as it has a positive predictive value and specificity of 98%.3 Additionally, the imaging can offer clinicians a visual representation of progress over time with treatment. While SGUS monitoring has its benefits, notable drawbacks include missing early disease if disease severity is mild, and US monitoring must be executed with the same technique, standardized scoring system, same machine, and technician if a long term follow-up is planned.

Sialendoscopy is a minimally invasive and effective tool for the diagnosis and treatment of glandular ductal pathology, including inflammation, stenosis, or stricture. In a review of juvenile recurrent parotitis, Schwarz et al note that it is unclear whether the steroid treatment or the procedure itself effectively treats symptoms.6 With that said, according to a study by Schiffer et al, patients with JSS can be diagnosed based upon sialendoscopy and histopathology from labial biopsy, and Faure et al note that sialendoscopy has a greater sensitivity than radiologic ultrasound or MRI.7 8 However, it should be noted that the findings of the sialendoscopy alone were not specific for diagnosis of JSS and other clinical indicators like labial biopsy or antibody titers were necessary for JSS diagnosis. For JSS, no reports to date adequately estimate the effectiveness of sialendoscopy in halting recurrence.

In pediatric populations, JSS will present more commonly with parotid involvement with punctate sialectasis in comparison to adults who can also have submandibular involvement and fatty degeneration.9 With this in mind, SGUS scoring may need to be modified to better suit the clinical distinctions of SS in the pediatric population. Whether sialendoscopy alone can provide a long-term remission of recurrent parotitis in patients with JSS warrants further investigation.

Objectives

The authors present the case of a 12 year-old female who was diagnosed with Juvenile Sjögren’s Syndrome (JSS) based on the 2016 ACR/EULAR SS diagnostic criteria for adults and monitored for progress via SGUS throughout treatment involving sialendoscopy.

This poster will discuss the nuances in diagnosing JSS through clinical presentation, ultrasound, and sialendoscopy.

This poster will discuss the nuances in the clinical presentation and diagnosis in Sjögren’s syndrome for the pediatric population in comparison to adults.