Abstract:

ABSTRACT Object: To investigate the clinical features and outcomes in AL amyloidosis patients with renal involvement.  Methodology: Two hundred and forty five patients with biopsy-proven AL amyloidosis in Jinling hospital from March 2003 to August 2011 were studied. They were 153 males and 92 females with the median age of 56 years old at diagnosis. All patients had renal involvement. The clinical and laboratory information were collected from diagnosis to death or until the last available clinical control. The patients’ survival and renal outcomes were analyzed, and the relationships between clinical parameters were also assessed. Results: The most common affect organs were intestinal tract (55.9%) and heart (46.7%) besides renal. Proteinuria was the major clinical feature of these patients, and the mean level of proteinuria was 4.86±4.32g/24hrs. 25% patients had renal insufficiency at diagnosis. The mean time of follow-up was 20 months (range from 1-98). 111 patients were dead, the median survival time of all patients was 33.6 months, the patients with heart involvement, hepatic involvement, hypotension and renal insufficiency had a worse survival, and specific therapy can improve the survival. The 1,2,3 and 5-year cumulative survival rate, calculated by Kaplan-Meier method, were 68.3%、52.7%、47.8% and 30.7% respectively. Multivariate COX analysis showed the age, hepatic involvement, heart involvement and specific therapy can significantly influence survival in these patients. The median time of renal survival was 45 months, the 1,2,3 and 5-year renal survival rate were 78.6%、69.6%、63.2% and 36.9% respectively, Multivariate COX analysis showed the levels of serum creatinine and degree of hypotension were the important risk factors of renal failure. Conclusion: Our data demonstrated the clinical features and survival of Chinese patients with renal AL amyloidosis. These patients were usually combined with intestinal tract and heart involvement, the survival of our cohort was poor, and the heart involvement, hepatic involvement, hypotension, renal insufficiency and specific therapy can significantly influence survival. The high serum creatinine level and hypotension at diagnosis implied the bad renal outcome.