1992 Volume 15 Issue 1 Pages 81-86
A case of hereditary angioneurotic edema (HANE) associated with Sjögren's syndrome is described. A 15-year-old female had been suffering from recurrent and sudden attacks of colicky abdominal pain since 11 years of age. Complement studies revealed low CH 50, C4 and C1 inhibitor levels, with normal C1q and C3 levels. No complement system defects were documented in other family members. However, normal C1 level, absence of lymphoproliferative diseases by gallium scintigram and undetectability of antibodies to C1 inhibitor suggest the patient suffered from HANE due to a spontaneous mutation of the C1-INH gene. She was also diagnosed as having Sjögren's syndrome from parotid sialogram findings and histological examination of minor salivary glands of the lip. Although several investigators have reported the cases of HANE associated with systemic lupus erythematosus, HANE associated with Sjögren's syndrome has not been fully documented.
