B cell malignant lymphoma in a patient with progressive systemic sclerosis and Sjögren's syndrome. Report of a case and review of the literature.

We report a patient with progressive systemic sclerosis (PSS) and Sjogren's syndrome (SS) who developed B cell malignant lymphoma 11 and 10 years after the onset of signs of PSS and SS, respectively. As her lymphoma developed, autoantibodies such as rheumatoid factor and antinuclear antibody disappeared from the serum, the lupus erythematosus test yielded negative results and her immunoglobulin levels declined. A good response to chemotherapy was obtained but she died of acute interstitial pneumonitis. Postmortem examination revealed marked diffuse interstitial fibrosis of the lungs.