Association of Sjögren's syndrome with hereditary angioneurotic edema: report of a case.

A case of hereditary angioneurotic edema (HANE) associated with Sjögren's syndrome is presented. One of the members of a pedigree of HANE due to deficiency of C1 inhibitor (C1INH) had a positive titer for anti-SS-A and anti-SS-B antibodies in the serum, complaining of symptom of dry eyes and dry mouth. A lip biopsy revealed lymphocytic infiltration of minor salivary glands. The patient had renal tubular acidosis (RTA). Thus the patient was diagnosed as suffering from Sjögren's syndrome with RTA.