西蒙布鲁姆注册研究中严重与非严重家族性高胆固醇血症患者的冠心病死亡率。
Coronary heart disease mortality in severe vs. non-severe familial hypercholesterolaemia in the Simon Broome Register.
机构信息
Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College London, University Street, London, WC1E 6JJ, UK.
Centre for Cardiovascular Genetics, Institute of Cardiovascular Science, University College London, University Street, London, WC1E 6JJ, UK.
出版信息
Atherosclerosis. 2019 Feb;281:207-212. doi: 10.1016/j.atherosclerosis.2018.11.014. Epub 2018 Nov 12.
BACKGROUND AND AIMS
The International Atherosclerosis Society (IAS) has proposed that patients with "severe" FH (SFH) would warrant early and more aggressive cholesterol-lowering treatment such as with PCSK9 inhibitors. SFH is diagnosed if LDL-cholesterol (LDLC) > 10 mmol/L, or LDLC >8.0 mmol/L plus one high-risk feature, or LDLC >5 mmol/L plus two high-risk features. Here we compare CHD mortality in SFH and non-SFH (NSFH) patients in the UK prospective Simon Broome Register since 1991, when statin use became routine.
METHODS
2929 definite or possible PFH patients (51% women) aged 20-79 years were recruited from 21 UK lipid clinics and followed prospectively between 1992 and 2016. The excess CHD standardised mortality ratio (SMR) compared to the England and Wales population was calculated (with 95% confidence intervals).
RESULTS
1982 (67.7%) patients met the SFH definition. Compared to the non-SFH, significantly (p < 0.001) more SFH patients had diagnosed CHD at baseline (24.6% vs. 17.5%), were current smokers (21.9% vs 10.2%) and had a BMI > 30 kg/m (14.9% vs. 7.8%). The SMR for CHD mortality was significantly (p = 0.007) higher for SFH (220 (184-261) (34,134 person years, 129 deaths observed, vs. 59 expected) compared to NSFH of 144 (98-203) (15,432 person years, 32 observed vs. 22 expected). After adjustment for traditional risk factors, the Hazard Ratio for CHD mortality in SFH vs. NSFH was 1.22 (0.80-1.87) p = 0.36, indicating that the excess risk was largely accounted for by these factors.
CONCLUSIONS
CHD mortality remains elevated in treated FH, especially for SFH, emphasising the importance of optimal lipid-lowering and management of other risk factors.
背景与目的
国际动脉粥样硬化学会（IAS）提出，患有“严重”家族性高胆固醇血症（SFH）的患者需要早期、更积极地进行降脂治疗，例如使用前蛋白转化酶枯草溶菌素 9（PCSK9）抑制剂。SFH 的诊断标准为 LDL-胆固醇（LDLC）＞10mmol/L，或 LDLC＞8.0mmol/L 且存在 1 项高危特征，或 LDLC＞5mmol/L 且存在 2 项高危特征。本研究旨在比较自 1991 年他汀类药物常规应用以来，英国前瞻性 Simon Broome 注册研究中 SFH 和非 SFH（NSFH）患者的冠心病死亡率。
方法
从英国 21 家血脂诊所招募了 2929 名确诊或可能的 PFH 患者（51%为女性），年龄为 20-79 岁，前瞻性随访 1992 年至 2016 年。采用 England 和 Wales 人群作为标准，计算冠心病超额标准化死亡率比（SMR）（95%置信区间）。
结果
1982 例（67.7%）患者符合 SFH 定义。与 NSFH 患者相比，SFH 患者基线时冠心病诊断率显著更高（24.6% vs. 17.5%）（p＜0.001），吸烟者比例显著更高（21.9% vs. 10.2%），BMI＞30kg/m2 的比例显著更高（14.9% vs. 7.8%）。SFH 患者的冠心病死亡率 SMR 显著更高（p=0.007），为 220（184-261）（34134 人年，129 例死亡，预期值为 59），而非 SFH 为 144（98-203）（15432 人年，32 例死亡，预期值为 22）。校正传统危险因素后，SFH 患者冠心病死亡的风险比为 1.22（0.80-1.87）（p=0.36），表明这些因素很大程度上解释了这种风险。
结论
FH 患者经治疗后冠心病死亡率仍较高，尤其是 SFH 患者，这突出了优化降脂治疗和管理其他危险因素的重要性。
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