A 52-year-old woman was admitted to our department because of fever and dysphasia in November 1994. She had noticed Raynaud's phenomenon, arthralgia, and stiffness in the skin for the past 20 years without receiving specific treatment. A diagnosis of progressive systemic sclerosis (PSS) was made based on the presence of sclerosis of the skin, sclerodactyly, pulmonary fibrosis, and the dilatation of the lower esophagus. It was also confirmed that systemic lupus erythematosus (SLE) overlapped based on the presence of an oral ulcer, polyarthralgia, leukocytopenia, renal dysfunction, positive tests for antinuclear antibodies and anti-DNA antibodies. A gastric biopsy specimen revealed amyloid deposits which showed green birefrigence by Congo red staining under polarized light microscopy. The disappearance of the green birefrigence after potassium permanganate treatment indicated that the deposits consisted of AA amyloid. No amyloid deposits were detected in the rectum or the kidney. The average of the SAA level in active SLE patients is 174 micrograms/ml in our department and the SAA level in the present case was 280 micrograms/ml. Although AA amyloidosis is rarely associated with SLE and PSS, a long-term elevation of SAA level could cause the development of amyloidosis even in the cases of SLE and PSS.