Sjogren Syndrome
Definition/Classification
Sjogren syndrome (SS) is a chronic autoimmune disease, of unknown etiology, whose main characteristic is lymphocytic infiltration of exocrine glands. SS may occur as an isolated disorder (primary) or, more commonly, in association with systemic lupus erythematosus or other connective tissue diseases. SS is characterized by a wide clinical spectrum ranging from isolated involvement of salivary and lacrimal glands to a systemic disease that may involve lungs, kidneys, muscles, and the central nervous system.
Epidemiology
Primary SS appears to be very rare in children, while secondary SS is more common. SS is more frequent in females and usually occurs in adolescence, although it has been observed in children as young as 5 years.
Pathology
The common finding in affected organs is lymphocytic infiltration. In salivary glands, infiltrating lymphocytes progressively replace the salivary epithelium. Germinal centers formation may occur. A typical...
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de Benedetti, F. (2012). Sjogren Syndrome, Raynaud’s Phenomenon, Overlap Syndromes. In: Elzouki, A.Y., Harfi, H.A., Nazer, H.M., Stapleton, F.B., Oh, W., Whitley, R.J. (eds) Textbook of Clinical Pediatrics. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02202-9_163
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