[An autopsied case of progressive systemic sclerosis with anti Wa antibody who showed a rapid progression].

An anti Wa antibody was reported as a new t-RNA related protein antibody in 1986. This autoantibody is now considered specific for the diagnosis of progressive systemic sclerosis (PSS). Up to date only 5 cases with anti Wa antibody have been identified. We report here an autopsied case of PSS with this antibody. A 53 years old female was admitted to our hospital because of dry cough and dyspnea in Sep 1987. There were fine crackles and chest X ray revealed interstitial pneumonia. The progressive respiratory failure was treated by steroid pulse therapy effectively. Sclerotic skin changes of hand began to appear in Sep 1988 and rapidly progressed to arms, chest and forehead by Dec 1988. A skin biopsy confirmed PSS changes. An anti Wa antibody was detected by double immunodiffusion and the protein antigen was associated with t-RNA when immunoprecipitation was conducted. She died of heart failure in July 1989. An autopsy revealed the diffuse fibrotic change of the heart and the lung. Cases with anti Wa antibody were shortly reviewed from the literature.