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Development of systemic sclerosis in a patient with systemic lupus erythematosus and topoisomerase I antibody

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We describe a patient with systemic lupus erythematosus (SLE) associated with topoisomerase I (topo I, Scl-70) antibody, a specific marker for systemic sclerosis (SSc). SSc patients who produce this antibody have severe cutaneous and visceral involvement, and eventually have a poor prognosis. It is rare to find this antibody in patients with other collagen diseases. Only four SLE patients have been reported in the English language literature who were topo I antibody-positive but had no clinical evidence of scleroderma. The serum of our patient with SLE had precipitating topo I antibody from the onset of his disease. Twelve years after the onset of SLE, he developed proximal scleroderma and pulmonary fibrosis. This case reconfirms the prognostic significance of topo I antibody as a predictive marker, and indicates that SLE patients with topo I antibody require careful follow-up for future development of scleroderma.
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Keywords: Scl-70 antibody; systemic lupus erythematosus; systemic sclerosis; topoisomerase I antibody

Document Type: Research Article

Affiliations: Department of Dermatology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8522, Japan

Publication date: May 1, 2000

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