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We present a unique case of familial primary Sjogren’s syndrome (pSS) involving a father and son that challenge several key features of this disease onset.
A 20-year-old man (fig 1) was referred with a 4-year history of recurrent parotitis and persistent bilateral parotid swelling. He also had increasing fatigue and night sweats for 3 months and cosmetically unacceptable parotid swellings. Although Schirmer’s test was normal, he was found to have positive Ro, La and antinuclear antibodies. Computerised tomography scans of his abdomen and chest showed no abnormality. Subsequent parotid biopsy showed a low-grade mucosa-associated lymphatic tissue (MALT) lymphoma. A diagnosis …
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