A case of sicca syndrome due to primary amyloidosis

We report the rare occurrence of sicca syndrome associated with primary amyloidosis. A 63-year-old man with apparent keratoconjunctivitis sicca and xerostomia died of the cardiac failure. Neither sialography nor labial gland biopsy revealed findings compatible with Sjögren's syndrome. Macroglobulinemia and a positive Bence-Jones protein were noted in urine testing. Amyloid deposition was demonstrated by Congo red staining on biopsied rectal tissues, and by typical birefringence under polarized light microscopy. At autopsy, the amyloid deposition was identified histochemically in the submandibular gland and the minor salivary glands of oral cavity.