Prognostic Factors and Survival
The histological hallmark of primary Sjögren’s syndrome (pSS) is the focal lymphocytic infiltrates that slowly and steadily replace the epithelium of salivary and lacrimal glands and produce exocrine gland dysfunction, manifested mainly by xerostomia and xerophthalmia . These two clinical manifestations are determinants for the term “exocrinopathy” in SS. Studies in the last 20 years have delineated the type and spectrum of other organ involvement in SS. Lungs, liver and the kidneys are usually affected in pSS. The histology and the evolution of the lesion in the above parenchymal tissues do not differ from that observed in salivary and lacrimal glands. These extragrandular manifestations are mild in severity in most cases, evolve slowly, and do not appear to respond to glucocorticoids or other immunosuppressive agents. The central role of the epithelial cell as the main target of the immune injury in the exocrine glands as well as in the lungs, the liver and the kidneys has led to the introduction of the term “autoimmune epithelitis” .
KeywordsInterstitial Lung Disease Lacrimal Gland Standardize Mortality Ratio Standardize Incidence Ratio Mucosa Associate Lymphoid Tissue Lymphoma
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