Abstract
A 65-year old Japanese woman was admitted to our hospital because of mental confusion and hemorrhagic tendency. She had a 10 years history of mild anemia and Raynaud's phenomenon, and also had xerostomia, xerophthalmia and oral aphthae since 1982. On admission, fever, delirious state, suggillations on the face, chest, arms, legs and abdomen, anemia and mild jaundice were observed. In addition, definite keratoconjunctivitis sicca and oral aphthae were seen. No lymphadenopathy nor hepatosplenomegaly was palpable. Laboratory examinations revealed microhematuria, anemia with fragmented red cells, increased indirect bilirubin and LDH and low level of haptoglobin indicating the presence of microangiopathic hemolytic anemia. Increased FDP, prolonged prothrombin time, low fibrinogen and severe thrombocytopenia clearly indicated the complication of DIC. Autoantibodies; antinuclear antibody, anti-DNA antibody, anti-ENA antibody, anti-RNP antibody, anti-microsoma antibody, antithyroglobulin antibody and elevation of the platelet associated IgG (Pa IgG) were detected, but LE-cell, anti-Sm antibody and coombs tests were negative. Histological examinations showed the deposition of IgG in dermoepidermal junction and fibrinogen in epidermis perivascular lesion of finger, mild lymphocyte infiltration in the microsalivery gland and vascular thrombas in the populus ulcer.
She was diagnosed as having autoimmune disease (SLE and Sjögren's Syndrome) and TTP-like disorder with DIC. High does of prednisolone, gabexate mesilate, fresh whole blood, and platelet consentrates were administered, and her symptomes were improved. The possible relationship between TTP with DIC and the autoimmune disorders was discussed.
