Abstract
Systemic Lupus Erythematosus (SLE) is associated with abnormal antibody production. Recent evidence also suggests T cell dysfunction in this disorder. The patient described had ante- and post-mortem evidence of thymic dysplasia.
A diagnosis of SLE was made in a 2 year old girl with clinical evidence of skin and joint involvement. Laboratory evidence included positive LE cells, high ANA titer, hypergammaglobulinemia and proteinuria. History of a severe reaction to smallpox vaccine suggested pre-existing T cell deficiency.
Excellent control of the disease was achieved with prednisone and cyclophosphamide for 7 months when progressive central nervous involvement began. Death occurred 1 year after diagnosis.
At autopsy the thymus was composed almost entirely of thymic epithelial cells. While depletion of lymphocytes and plasma cells may be explained by prolonged immunosuppressive therapy, the total absence of Hassall's corpuscles suggests thymic dysplasia. In this patient, there may be an abnormity of T cell modulation of B cell function resulting in formation of antibodies to non-foreign antigens.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
McWilliams, N., Jones, J., Joshi, V. et al. THYMIC DYSPLASIA IN A CHILD WITH SYSTEMIC LUPUS ERYTHEMATOSUS. Pediatr Res 8, 415 (1974). https://doi.org/10.1203/00006450-197404000-00450
Issue Date:
DOI: https://doi.org/10.1203/00006450-197404000-00450