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Clinical Features and Outcomes of Renal Amyloidosis: An Analysis of 35 Patients in a Single Taiwan Center
並列摘要
BACKGROUND: Renal amyloidosis is an unusual diagnosis in patients undergoing kidney biopsy. Knowledge of the clinical features and long-term outcomes is still very limited. The current study represents our single-center experience in the past 27 years. METHODS: Six thousands renal biopsies were performed between January 1983 and June 2010 at Taichung Veterans General Hospital. Of them, 720 cases were from renal allograft and 5280 were from native kidneys. A retrospective study via chart review was conducted to identify cases of renal amyloidosis. The clinical features as well as laboratory data at presentation and subsequent follow-up were retrieved and analyzed. RESULTS: Among 5280 cases of native kidney biopsies, 35 renal amyloidosis (0.66%) were identified. The mean follow-up duration is 1155 days. The incidence of renal amyloidosis among proteinuric patients appeared to be increasing. The male to female ratio was 24:11. Seventeen (48.6%) cases were of light chain amyloidosis (AL) type while the others were of non-AL types. The mean age was 61.7 ± 12.9 years, while the duration between apparent onset and kidney biopsy was 299.4 ± 312.2 days. Most of the cases (83.9%) had nephrotic-range proteinuria. The mean 24-hour urine protein was 7.4 ± 4.7 g (median: 6.48 g/day, 2.13-22.9 g/day), serum albumin, 2.39 ± 0.7 g/dL; total cholesterol, 327.5 ± 144 mg/dL; and triglyceride, 223.7 ± 150 mg/dL. Hematuria occurred in 40% of the cases. The eGFR at renal biopsy was 56.3 ± 28.2 mL/min. There was no obvious enlargement in renal size (right kidney: 102.7 ± 15.6 mm, left kidney: 107.9 ± 14.2 mm). The one-year and five-year patient survival was 42.9% and 17.9%, respectively. Logistic regression analysis revealed that eGFR was the only predictive factor for patient survival. CONCLUSION: Elderly patients with nephrotic syndrome should prompt the suspicion of renal amyloidosis, especially in those with nephritic syndrome and relatively low blood pressure. Given the increasing incidence and poor prognosis, early diagnosis and a search of the underlying etiology of amyloidosis are mandatory. (Acta Nephrologica 2011; 25: 113-118)