| Summary points: | A 64-year-old Caucasian female with primary Sjögren syndrome presents with dry mouth, eyes, and vagina, as well as intermittent swelling of the salivary glands and aching joints. She had given birth to 2 sons with congenital heart block, both of whom required pacing several years later. Current medications, medical history, extraoral examination, and blood tests are described. The authors discuss the association between Sjögren syndrome and congenital heart block, the suggested mechanism, the long-term outcome of mothers of children with congenital heart block, and preventive treatment strategies. |