Department of Medicine and Geriatrics, Caritas Medical Centre, Kowloon, Hong Kong.
Department of Medicine and Geriatrics, Yan Chai Hospital, New Territory, Hong Kong.
Nephrology (Carlton). 2022 Nov;27(11):869-876. doi: 10.1111/nep.14089. Epub 2022 Aug 25.
Early symptoms of primary (AL) amyloidosis are non-specific. Any delay in diagnosis and treatment results in poor outcome despite increasing treatment options. We aimed to determine baseline risk factors that identify patients with poor kidney outcomes and overall survivals.
We recruited all patients aged 18 years or above with biopsy-proven renal amyloidosis between years 2000 and 2019 in three Hong Kong regional hospitals. Patients' clinical and pathological parameters, treatment response, kidney outcomes and overall survivals were recorded and analysed.
Thirty-six cases of renal amyloidosis were recruited. Four cases were diagnosed to have multiple myeloma. Edema was the most common presenting symptom. The mean estimated glomerular filtration rate (eGFR) was 98.8 ml/min/1.73 m at presentation. Autologous stem cell transplant conferred the best renal outcomes as well as patients' survival. Twenty-two patients had 50% decrease in eGFR, 12 patients developed end-stage kidney disease (ESKD) and 22 patients died. Hypertension, diabetes mellitus, proteinuria and low eGFR were identified as independent baseline risk factors for ESKD. Proteinuria, hyperlipidemia, and cardiac involvement were independent baseline risk factors for death.
Amyloidosis, a rare disease with poor prognosis without treatment. Hypertension, diabetes mellitus, heavy proteinuria and low eGFR at diagnosis were associated with poor kidney outcome.
原发性（AL）淀粉样变性的早期症状是非特异性的。尽管治疗选择越来越多，但任何诊断和治疗的延迟都会导致预后不良。我们旨在确定基线风险因素，以识别具有不良肾脏结局和总体生存率的患者。
我们招募了 2000 年至 2019 年间在香港三家地区医院通过活检证实的年龄在 18 岁或以上的所有肾淀粉样变性患者。记录并分析了患者的临床和病理参数、治疗反应、肾脏结局和总体生存率。
共招募了 36 例肾淀粉样变性患者。4 例被诊断为多发性骨髓瘤。水肿是最常见的首发症状。就诊时平均估算肾小球滤过率（eGFR）为 98.8ml/min/1.73m。自体干细胞移植对肾脏结局和患者生存均有最佳效果。22 例患者 eGFR 下降 50%，12 例患者发展为终末期肾病（ESKD），22 例患者死亡。高血压、糖尿病、蛋白尿和低 eGFR 是 ESKD 的独立基线危险因素。蛋白尿、高脂血症和心脏受累是死亡的独立基线危险因素。
淀粉样变性是一种预后不良的罕见疾病，如果不治疗，预后更差。诊断时的高血压、糖尿病、大量蛋白尿和低 eGFR 与不良肾脏结局相关。
