Dense Deposit Disease Associated With Monoclonal Gammopathy of Undetermined Significance

摘要: Dense deposit disease (DDD) is a rare glomerular that typically affects children, young adults, and much less commonly, older patients. The pathophysiologic process underlying DDD uncontrolled activation of the alternative pathway (AP) complement cascade, most frequently secondary to an autoantibody C3 convertase called nephritic factor, although mutations in factor H autoantibodies this protein can impair its function also cause DDD. Since 1995, we have diagnosed 14 patients aged 49 years or older; 10 these (71.4%) carry concomitant diagnosis monoclonal gammopathy undetermined significance (MGUS). In 1 patients, index case described here, evaluated AP showed low serum levels consistent with activity, heterozygosity for H402 allele H, autoantibodies, which affect ability regulate activity. aggregate, findings suggest some adults MGUS, may develop as result (or other proteins) on permissive genetic background (the H) lead dysregulation subsequent damage. Thus, be distinct clinicopathologic entity represents uncommon complication MGUS.