Primary amyloidosis presenting as Sjögren's syndrome

A patient had severe sicca complex. Histopathologic and immunologic examination of the salivary glands showed amyloid fibril infiltration, type A lambda VI. To our knowledge, this is the first documentation of immunologically characterized primary amyloidosis causing the sicca complex. The sicca complex is usually associated with Sjögren's syndrome and the presence of autoantibodies to SSA and SSB. These antibodies were absent in our patient, despite the severity of the sicca syndrome. The clinician should consider more unusual causes of the sicca complex, eg, amyloidosis, particularly if the serologic markers of antibodies to SSA and SSB are absent. Tissue typing the amyloid protein diagnosed primary amyloidosis, obviating the need to search for underlying disease.