We describe a patient who has primary Sjogren's syndrome associated wi
th asymptomatic gamma heavy chain disease and a tubulointerstitial nep
hritis. Sjogren's syndrome is known to be complicated by lymphoprolife
rative disorders and tubulointerstitial nephritis but gamma heavy chai
n disease is Fare (approximately 100 cases described). There is one pr
eviously reported case of gamma heavy chain disease associated with pr
imary Sjogren's syndrome and 2 cases associated with secondary Sjogren
's syndrome. Our patient and the 3 other patients described in the lit
erature did not have evidence of an underlying lymphoproliferative dis
order.