2018 Volume 30 Issue 4 Pages 276-283
A man in his sixties was referred to our hospital with numbness in the extremities and presented with systemic lymph node swelling including prominent immunoglobulin(Ig)G4-positive cell infiltration as well as an elevated serum IgG4 level. He lacked subjective sicca symptoms but was positive for anti-SS-B antibody. Periductal focal mononuclear cell infiltration in labial salivary gland biopsy specimen led to the diagnosis of Sjögren’s syndrome. This is the first case of a patient with anti-SS-A antibody-negative/anti-SS-B antibody-positive Sjögren’s syndrome concomitant with increased serum and lymph node IgG4.