Abstract
An autopsy case of a 44-year-old woman with mixed connective tissue disease (MCTD) is presented. Clinically, PSS-like manifestations were outstanding and the entire course of illness was 14 years. The patient died of cerebral hemorrhage. Autopsy disclosed widespread scleroderma, hypertensive pulmonary vascular disease, and nodular regenerative hyperplasia (NRH) of the liver. Relationships between NRH and collagen diseases including MCTD, drugs administered, portal and pulmonary hypertension are discussed.
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