Abstract
Two patients, a mother and daughter, each with the CRST syndrome, developed Sjögren's syndrome. Both patients had mitochondrial antibodies, smooth muscle antibodies, and a raised serum IgM without clinical evidence of liver disease. This family constitutes the first record of the familial coexistence of the CRST syndrome with Sjögren's syndrome, and the second evidence of vertical inheritance of scleroderma. It is suggested that patients with the CRST syndrome be studied for Sjögren's syndrome and for autoimmune liver disease.