Renal involvement in XXXX : an Italian collaborative study on survival and renal outcome.

@entity1 @entity644 @entity8 @entity5066 @entity2783 @entity1582 @entity1011 @entity204 @entity454
BACKGROUND: Few data are available from large population-based studies on survival and renal outcome of @entity1 with renal involvement and different types of @entity1582 . METHODS: Two hundred and ninety of over 373 @entity1 affected from @entity1582 with renal involvement diagnosed in Italy between January 1995 and December 2000 were followed from diagnosis to @entity204 or until the last available clinical control. Eighty-three @entity1 were excluded from analysis either because the amyloid type remained undetermined or they were lost at follow-up. Clinical and laboratory information was collected according to the different types of @entity2783 using a specific form which included renal function with 24 h @entity644 at diagnosis and at the end of follow-up, the type and the date of onset of dialysis and the kind of treatment they underwent. RESULTS: The median time of follow-up was 24 months in primary ( @entity1582 ) @entity2783 (range: 1-88 months), 16 months in @entity1582 with associated @entity1582 (MM + @entity1582 : range 1-76 months), 30 months in reactive ( @entity5066 ) @entity2783 (range: 1-99 months) and 52 months in @entity1 with familial forms (AF: range 14-82 months). @entity1 with @entity1582 showed a significantly shorter survival than @entity5066 . Despite no significant differences of renal outcome or survival on dialysis being observed between the two groups, a lower renal survival with a higher number of @entity1 who progressed to @entity454 ( @entity454 ) was observed in @entity1 with @entity5066 . Overall survival was markedly improved in @entity1 with @entity1582 who underwent a specific therapy (conventional chemotherapy or autologous stem cell transplantation (ASCT)) even in the absence of a positive kidney response. Multivariate analysis showed cardiac involvement and specific therapy to significantly influence survival in @entity1582 whereas age, serum @entity1011 (sCr) and heart involvement significantly affected survival in @entity5066 . In both groups, sCr and heart involvement were the most relevant predictors for renal outcome, together with urinary protein excretion, in @entity1 with @entity5066 . CONCLUSIONS: Our results show a worse survival in @entity1582 due to the higher prevalence of heart involvement in this group and emphasize that a specific therapy significantly prolongs survival and slows the progression of @entity8 in @entity1 with @entity1582 . We suggest that a late nephrological referral is likely the cause of the higher sCr found at presentation in @entity1 with @entity5066 and probably accounts for the lower renal survival observed in the short term in these @entity1 . At the time being, renal transplantation and ASCT are still rare therapeutic options for renal @entity1 affected from @entity1582 .
