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Autoimmune epitheliitis: Sjögren's syndrome
F N Skopouli1, H M Moutsopoulos
1Department of Medicine, School of Medicine, Ioannina, Greece.
Clinical and Experimental Rheumatology
|November 1, 1994
Summary
Sjögren's syndrome (SS) involves autoimmune attacks on epithelial tissues, causing kidney, lung, and liver dysfunction. Researchers propose renaming it "Autoimmune Epitheliitis" to reflect the primary epithelial tissue involvement.
Area of Science:
- Immunology
- Pathology
- Rheumatology
Background:
- Sjögren's syndrome (SS) is a common autoimmune exocrinopathy primarily affecting females.
- Clinical manifestations include kidney, lung, and liver involvement, often presenting as interstitial nephritis, subclinical interstitial lung disease, and pericholangeal infiltrate, respectively.
Purpose of the Study:
- To investigate the underlying mechanisms of systemic manifestations in Sjögren's syndrome.
- To propose a new terminology, "Autoimmune Epitheliitis," to better describe the disease pathology.
Main Methods:
- Clinical evaluation of kidney, lung, and liver involvement in SS patients.
- Histopathological and molecular studies of minor salivary gland epithelial cells.
- In situ hybridization to assess cytokine expression (IL-1, IL-6).
Main Results:
- Epithelial cells in SS patients inappropriately express HLA class II molecules and c-myc.
- Pro-inflammatory cytokines IL-1 and IL-6 are produced by epithelial cells.
- Proviral DNA was found incorporated into the DNA of epithelial cells.
Conclusions:
- Systemic manifestations of SS are likely driven by lymphocyte attraction to epithelial tissues.
- The epithelium is the primary target tissue in the autoimmune histopathological lesions of SS.
- The term "Autoimmune Epitheliitis" is proposed to accurately represent the disease's core pathology.