J Rheum Dis.  2024 Oct;31(4):230-243. 10.4078/jrd.2024.0043.

Clinical characteristics of chronic sclerosing sialadenitis as a distinctive entity from primary Sjögren’s syndrome

• Kwon EJ
• Park Y
• Kwok SK
• Ju JH

• KMID: 2559892
• DOI: http://doi.org/10.4078/jrd.2024.0043

Abstract

Objective
This study aimed to elucidate the clinical and laboratory differences between chronic sclerosing sialadenitis (CSS) and primary Sjögren’s syndrome (pSS), highlighting CSS as a distinct pathological entity within the spectrum of salivary gland pathology.
Methods
This retrospective, single-center study was conducted at Seoul St. Mary’s Hospital between January 2000 and December 2022. Patients diagnosed with CSS via salivary gland biopsy were included, and those with IgG4-related disease (IgG4-RD) or other confounding factors were excluded. Clinical and laboratory CSS profiles were compared with those of a control group of patients with typical pSS from the Korean Initiative of Primary Sjögren’s Syndrome (KISS) prospective cohort study. Twenty-one with CSS and 501 patients with pSS from Seoul St. Mary’s Hospital were retrospectively analyzed.
Results
Patients with CSS were older at diagnosis, had a lower prevalence of ocular symptoms, and exhibited distinct immunological markers compared to those with pSS. Logistic regression analysis revealed that anti-Ro antibody positivity, elevated erythrocyte sedimentation rate levels, low serum complement 3 levels, and accompanying dry eye symptoms were factors distinguishing pSS from CSS.
Conclusion
Even after excluding IgG4-RD, CSS was significantly different from pSS in terms of clinical and laboratory findings. Recognition of these differences is crucial for the accurate diagnosis and management of CSS, underscoring its status as a distinct pathological entity among salivary gland pathologies.

Keyword

Chronic sclerosing sialadenitis; Küttner’s tumor; Sjögren’s syndrome; Immunoglobulin G4-related disease

Figure

• 

  Figure 1 Schematic representation of study population composition. Total 260 patients (21 of CSS group and 239 of typical pSS group from KISS cohort) were enrolled. CSS: chronic sclerosing sialadenitis, pSS: primary Sjögren’s syndrome, IgG4: immunoglobulin G4, KISS cohort: Korean Initiative of Primary Sjögren’s Syndrome cohort, ACR: American College of Rheumatology, EULAR: European Alliance of Associations for Rheumatology.

• 

  Figure 2 Result of all-subset regression. Utilizing the imputed dataset, an all-subset regression analysis was conducted to identify variables with the highest explanatory power. The horizontal line represents the variables in all-subset regression. The vertical line indicates the value of R square. Known interactions between ESR and hematocrit, as well as between WBC count and absolute neutrophil count, were taken into consideration within the framework of the all-subset regression analysis. In the context of the all-subset regression, ESR and ANC were chosen as variables for analysis, while acknowledging their interactions with Hct and WBC, respectively. Additionally, during the all-subset regression analysis, the interaction between ESR and age at diagnosis was also incorporated into the analysis. Age: age at diagnosis, Eye: ocular symptoms, Ro: anti-Ro antibody positivity, C3: low serum C3, EGM: extraglandular manifestation, RF: rheumatoid factor, WBC: leukopenia, ESR: erythrocyte sedimentation rate, glc: serum fasting glucose level, TC: serum total cholesterol.

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