GLOMERULONEPHRITIS ASSOCIATED WITH COMPLETE DEFICIENCY OF THE 4TH COMPONENT OF COMPLEMENT - RESPONSE TO INTRAVENOUS IMMUNOGLOBULIN

A 15-year-old girl with complete C4 deficiency and a lupus-like disord er developed evidence of nephritis after 4 years of followup, Renal bi opsy demonstrated an immune complex glomerulonephritis, with deposits in the capillary loops, the paramesangium, and the mesangial matrix, R enal function was normal, The patient was treated with monthly infusio ns of intravenous immunoglobulin for 6 months, The treatment was well tolerated, and resulted in resolution of the rash and hematuria. Follo wup biopsy showed less proliferation and fewer loop deposits. In light of the serious risk of infections that is associated with complement deficiency, approaches to glomerulonephritis that do not include immun osuppression should be considered.