Progressive systemic sclerosis is a connective tissue disease of unknown aetiology. This is the first study to demonstrate induction by a human B cell line of IL-6 secretion from fibroblasts. The cell line was established from lesional lung tissue of a patient with progressive systemic sclerosis. These cells, referred to as kon-1 cells, showed characteristics of pro-B cell by flow cytometry. Although kon-1 cells alone secreted a small amount of IL-6, a co-culture of kon-1 cells with normal lung fibroblasts significantly increased IL-6 levels. Whereas IL-6 mRNA was weakly expressed in kon-1 cells alone, it was clearly expressed in cells from the co-culture. Immunocytochemical identification of IL-6 showed localization in the cytoplasm of fibroblasts. IL-6 is a pleiotropic cytokine, essential for B cell differentiation, which has been shown to stimulate the production of collagen and glycosaminoglycan. Thus, abnormally augmented B cell proliferation and the inflammatory response stimulated by these cells may cause the fibrotic changes in patients with progressive systemic sclerosis.