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Chronic Sclerosing Sialadenitis of the Submandibular Gland and its Histopathological Spectrum in the IgG4-Related Disease: a Series of 17 Cases

  • Vinícius Rio Verde Melo Muniz,
  • Albina Altemani,
  • Valéria Souza Freitas,
  • Bruno Cunha Pires,
  • Dandara Andrade de Santana,
  • Larissa Abbehusen Couto,
  • Maria Cristina Teixeira Cangussu,
  • Ricardo Santiago Gomez,
  • Suzana Catanhede Orsine Machado de Souza,
  • Pablo Augustin Vargas,
  • Patrícia Ramos Cury,
  • Iguaracyra Barreto de Araújo,
  • Roberta Rayra Martins Chaves,
  • Felipe Paiva Fonseca,
  • Jean Nunes dos Santos

摘要

Purpose

This study aimed to characterize the histopathological immunohistochemical features of chronic sclerosing sialadenitis, emphasizing the IgG4-related disease.

Methods

Seventeen cases of chronic sclerosing sialoadenitis were examined for histopathological aspects, (inflammation, fibrosis, glandular parenchyma, and lymphoid follicles) and immunohistochemistry (BCL2, CD3, CD20, CD34, CD163, p63, cyclin D1, mast cell, SMA, S100A4, IgG, and IgG4) which were scored. IgG4-related disease features were investigated. Demographic and clinical data were also collected.

Results

Males predominated (10:7), with an average lesion size of 3.9 cm. Common histopathological findings included reduced acinar parenchyma, lymphoid follicle formation, and ductular proliferation. CD3-positive T lymphocytes and CD34- and SMA-positive stromal fibroblasts were abundant. Nine cases (53%) showed sialoliths and three cases met the criteria for IgG4-related disease.

Conclusion

CSS of the submandibular gland represents a reactive pattern rather than IgG4-RD as only 3 cases seemed to be related to IgG4-RD. The immunohistochemical profile revealed an abundant population of CD3-positive T lymphocytes, as opposed to regulatory proteins such as cyclin D1, demonstrating that populations of CD34- and SMA-positive stromal fibroblasts contribute to the fibrosis characteristic of CSS. In addition, our results provide a comprehensive insight into the study of CSS and its relationship with IgG4-RD.