Sjögren's syndrome with marked swelling of major salivary glands related to localized AL amyloidosis: A case report and literature review

Sjögren's syndrome (SS) is commonly observed in Japan, and oral surgeons perform lip biopsy and other examinations for definite diagnosis. Oral and maxillofacilal region are not the site of predilection for localized AL amyloidosis. In addition to that, SS related to localized AL amyloidosis is extremely rare.Here we report a rare case of SS associated with localized AL amyloidosis that caused marked swelling of bilateral submandibular and parotid glands. A 50-year-old man became aware of dry mouth, dry eyes and swelling of bilateral submandibular regions, and was referred to our hospital for definitive diagnosis in August 2008. Although clinical findings were not necessarily typical for SS, open biopsy of the submandibular gland and systemic examinations confirmed SS with localized AL amyloidosis. In the literature, SS could occur along with amyloidosis, so staining for amyloidosis in the biopsy specimen and systemic examination would be useful for this kind of disease.