Systemic light chain amyloidosis and Sjogren syndrome: an uncommon association.

Abstract : Sjogren syndrome is associated with lymphoproliferative disease in 7% of cases; however, association with AL amyloidosis is uncommon. We present a patient who presented simultaneously with Sjogren syndrome (supported by dry mouth, positive Schirmer's test, anti-Ro/SSA antibodies, and a lower lip salivary gland biopsy) and AL amyloidosis revealed by heart failure without myeloma. Although is it know that amyloidosis can masquerade as Sjogren syndrome, the occurrence of simultaneous AL amyloidosis and primary Sjogren syndrome has been reported rarely.