Systemic lupus erythematosus manifested after five years follow-up of hypergammaglobulinemia associated with systemic lymphadenopathy.

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A case in whom systemic lupus erythematosus (SLE) became evident five years after the appearance of lymphadenopathy and polyclonal hypergammaglobulinemia, is reported. The patient is a 33-year-old woman in whom hypergammaglobulinemia was incidentally pointed out 5 years ago. She had no symptoms and small lymphnodes were detected at the left side of the neck. LE cell phenomenon was negative but anti-nuclear antibody and anti-DNA antibody were weakly positive. Lymphangiography revealed significant swelling of the lymph nodes in the bilateral inguinal and paraaortic regions. Histologically, plasma cell hyperplasia was evident, the finding compatible with idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL). Five years later she developed alopecia, high fever and morning stiffness. LE cell phenomenon became positive and the titers of the anti-DNA antibody and anti-nuclear antibody markedly increased. According to the criteria for SLE of ARA (1982), a diagnosis of SLE was made.This case represented a subclinical on latent stage of SLE manifested by lymphadenopathy and hypergammaglobulinemia and may give some ideas to consider the mechanisms of the development of SLE.
日本臨床免疫学会の論文