XXXX : a report of 66 cases from a single institution.

@entity1 @entity644 @entity8 @entity101 @entity2743 @entity5 @entity1252 @entity189 @entity30 @entity177 @entity1011 @entity1985 @entity3039 @entity811 @entity631 @entity792 @entity454
BACKGROUND AND OBJECTIVES: @entity631 ( @entity631 ) is a rare primary @entity8 . Most previously reported cases were idiopathic. To better define the clinical-pathologic spectrum and prognosis, we report the largest single-center series with the longest follow-up. DESIGN, SETTING, @entity1 , _ MEASUREMENTS: The characteristics of 66 @entity631 @entity1 who were seen at Mayo Clinic, Rochester, between 1993 and 2010 are provided. RESULTS: The mean age at diagnosis was 53 years. Ninety-five percent of @entity1 were white, and the female:male ratio was 1.2:1. Underlying @entity5 (most commonly @entity189 ), dysproteinemia, or @entity792 (most commonly @entity30 , @entity1252 , @entity2743 , and @entity3039 ), were present in 23, 17, and 15% of @entity1 , respectively. Presentation included @entity644 (100%), @entity1985 (38%), @entity177 (66%), @entity811 (52%), and @entity101 (71%). The most common histologic pattern was mesangial proliferative/sclerosing GN followed by membranoproliferative GN. During an average of 52.3 months of follow-up for 61 @entity1 with available data, 13% had complete or partial remission, 43% had persistent @entity8 , and 44% progressed to @entity454 . The disease recurred in 36% of 14 @entity1 who received a kidney transplant. Independent predictors of @entity454 by multivariate analysis were older age, higher @entity1011 and @entity644 at biopsy, and higher percentage of global @entity631 . CONCLUSIONS: Underlying @entity5 , dysproteinemia, or @entity792 are not uncommon in @entity1 with @entity631 . Prognosis is poor, although remission may occur in a minority of @entity1 without immunosuppressive therapy. Age, degree of @entity8 at diagnosis, and degree of glomerular scarring are predictors of renal survival.
