Evidence for autoimmunity in the tight skin mouse model of systemic sclerosis

The tight skin mouse strain has been proposed for use as an animal model of systemic sclerosis because this animal exhibits a condition that has biochemical and pathologic similarities to the human disease. To date, however, evidence of inflammatory and immunologic changes in the tight skin mouse has been scarce. We demonstrated the presence of antinuclear antibodies in approximately half of these mice ages 8 months and older. This suggests that there is an autoimmune component in their disease process. The antibodies were identified as anti–topoisomerase I by a characteristic staining pattern on HEp-2 cells and by Western blot analysis. Except for a low incidence of anti-DNA antibodies, none of the other parameters tested, including mitogen responses, lymphokine production, and anti-erythrocyte antibodies, was indicative of immune system dysregulation.