[An autopsied case of PSS associated with Sjögren's syndrome and secondary generalized amyloidosis]

Fifty year-old female with progressive systemic sclerosis associated with Sjögren's syndrome who died of meteorism, diarrhea, bleeding of digestive canal due to amyloid deposition in the intestine and jaundice was reported. Autopsy revealed pathological findings compatible with that of PSS and Sjögren's syndrome. Furthermore, deposition of amyloid materials were shown in tissue of digestive canal, thyroid gland, heart muscles, lymphnodes, and small vessel wall of the whole body. Amyloid material was demonstrated to be A-A protein based upon the loss of Congo red stain after exposure to potassium permanganate, and reaction to anti A-A protein antibody. Although obstruction of intrahepatic bile duct, especially in Hering canal, could be relating to some unknown factors secondary to intestinal amyloidosis, further studies will be required to clarify the pathogenesis of it.