Aortitis syndrome associated with Sjögren's syndrome: Report of an autopsy case

Abstract

We described an autopsy case of aortitis syndrome associated with Sjögren's syndrome. A 52 years-old female was admitted to our hospital with adult distress syndrome (ARDS). She had been diagnosed as aortitis syndrome, Sjögren's syndrome and pulmonary fibrosis from March 1985. She died of pulmonary hemorrage due to miliary tuberculosis. Autopsy revealed that 1) arteritis affecting the aortic arch and subclavian artery, 2) chronic siliatis in mandibular glands, 3) pulmonary fibrosis, 4) miliary tubercules in the multiple organs. Although certain rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and Behcet's disease have been reported to be associated with aortitis syndrome, this is a first case report of a patient with aortitis syndrome associated with Sjögren's syndrome.