Analysis Of Clinical Characteristics, Renal Outcome And Prognosis Of Patients With Renal Involvement Of Light Chain Amyloidosis

Background:Immunoglobulin light-chain(AL)amyloidosis is the most common and serious type of systemic amyloidosis,with multiple organs and tissue involvement as the main clinical features,eventually causing life-threatening organ failure.However,most studies only focused on the overall survival of AL amyloidosis.Renal involvement is one of the most common complications of the disease,and can lead to end-stage renal disease(ESRD),which limits the therapeutic options and impact life quality.Our study compared clinical features of the amyloidosis cohort in multiple aspects,and emphasized on renal outcomes and survival outcomes.And we also aimed to validate two staging system for renal involvement in Asian populations firstly.Methods:Patients with newly diagnosed AL amyloidosis and kidney involvement which was proved by renal biopsy in Beijing Union Medical College Hospital from January 1,2002 to December 31,2017 were included.Demographic characteristics and clinical features were summarized.Cases diagnosed after 2011 were followed up.A comparative analysis of the baseline condition and prognosis was made between primary amyloidosis and secondary amyloidosis,? type and ? type.Two renal staging system were proposed in 2014 and 2017,based on proteinuria(24hUP>5g/d)and eGFR(<50ml/min/1.73m2)or 24hUP/eGFR at diagnosis.Patients were divided into renal stage ?-? and renal stage 1-3 respectively by two criteria.The baseline conditions and renal outcomes were analyzed and compared to evaluate the prognostic value of the two systems.Results:A total of 76 cases were included.All cases had renal involvement.44 cases were followed up,of which 11 were lost.The median follow-up time was 12.5 months.49 cases(64.5%)showed nephrotic syndrome,and 28 cases(36.80%)showed renal insufficiency at the time of diagnosis.There was no significant difference in clinical features,organ involvement renal outcome and survival between ? and ? cases,primary AL amyloidosis and AL amyloidosis secondary to multiple myeloma.The renal staging system based on 24hUP and eGFR were confirmed.There was a significant difference in renal outcome among renal stage ?-?(P=0.003).Based on the 24hUP/eGFR system,there was no ESRD in both stage 1 and stage 2.A significant difference was seen between phase 3 and two other stages(P=0.008).Conclusion:The clinical manifestations,renal outcome,and survival of AL amyloidosis are not affected by primary or secondary multiple myeloma,and different type of light chain.For the first time,the prognostic value of renal staging system based on 24hUP and eGFR was validated in Asian populations,which is of great significance in guiding clinical treatment and disease evaluation in AL amyloidosis.