Coexistance of Amyloidosis and Primary Sjögren's Syndrome: An Overview

Background: The association of primary Sjogren´s Syndrome (SS) and amyloidosis has been recognized but scarcely assessed. Objective: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. Methods: PubMed and CINAHL databases were searched from inception until April 2016 for the keywords: Amyloidosis, amyloid, Sjögren's syndrome and their combinations. Results: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic. Other places of localized amyloidosis were the kidney, tracheobronchial, tongue, breast, salivary gland and vocal cord. Most of the localized forms corresponded to AL type, whereas AA amyloidosis was uncommon. The diagnosis of amyloidosis followed the SS onset (1-25 years) and in the majority of patients the presence of B cell hyperactivity traduced by hypergammaglobulinemia, positive rheumatoid factor and/or anti-Ro/SSA and anti-La/SSB antibodies were observed. In seven patients, an associated lymphoma was also documented. Conclusion: The spectrum of lymphoid proliferation associated with SS extends beyond the classical B cell lymphoma. Localized or systemic amyloidosis might coexist with primary SS. Localized amyloidosis should be suspected in patients with consistently high serologic activity and suggestive lesions.