Institute for Cardiac Diagnostics and Therapy (IKDT), Berlin, Germany.
Department of Cardiology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Eur J Heart Fail. 2018 Apr;20(4):751-757. doi: 10.1002/ejhf.1039. Epub 2017 Oct 25.
To evaluate the influence of endomyocardial biopsy (EMB)-proven intramyocardial inflammation on mortality in patients with cardiac transthyretin amyloid (ATTR) or amyloid light-chain (AL) amyloidosis.
We included 54 consecutive patients (mean age 68.83 ± 9.59 years; 45 men) with EMB-proven cardiac amyloidosis. We followed up patients from first diagnostic biopsy to as long as 36 months (mean 11.5 ± 12 months) and compared their outcome with information on all-cause mortality with or without proof of inflammation on EMB. Intramyocardial inflammation was assessed by quantitative immunohistology. Patients suffering from amyloidosis revealed a significant poor prognosis with proof of intramyocardial inflammation in contrast to those without inflammation (log-rank P = 0.019). Re-grouping of patients indicated AL amyloidosis to have a significant impact on all-cause mortality (log-rank P = 0.012). The detailed subgroup analysis showed that patients suffering from AL amyloidosis with intramyocardial inflammation have a significantly worse prognosis compared with AL amyloidosis without inflammation and ATTR with or without inflammation, respectively (log-rank P = 0.014, contingency Fisher's exact test, P = 0.008).
Our study reports for the first time a high incidence (48.1%) of intramyocardial inflammation in a series of patients with EMB-proven cardiac amyloidosis and could show that in patients with AL amyloidosis, intramyocardial inflammation correlated significantly with increased mortality. Our data have a direct clinical impact because one can hypothesize that additional immunomodulating/anti-inflammatory treatment regimens in patients with biopsy-proven inflammation of heart muscle tissue could be beneficial for patients suffering from cardiac AL amyloidosis.
评估心肌内炎症对心肌活检（EMB）证实的心脏转甲状腺素蛋白（ATTR）或轻链（AL）淀粉样变患者死亡率的影响。
我们纳入了 54 例连续的 EMB 证实的心脏淀粉样变患者（平均年龄 68.83±9.59 岁；45 名男性）。我们对患者进行了从首次诊断性活检到最长 36 个月的随访（平均 11.5±12 个月），并将他们的预后与 EMB 证实的炎症或无炎症的全因死亡率信息进行了比较。通过定量免疫组化评估心肌内炎症。与无炎症的患者相比，有心肌内炎症的淀粉样变患者预后明显较差（对数秩检验 P=0.019）。重新分组后，AL 淀粉样变对全因死亡率有显著影响（对数秩检验 P=0.012）。详细的亚组分析表明，与无炎症的 AL 淀粉样变和有或无炎症的ATTR 相比，有心肌内炎症的 AL 淀粉样变患者的预后明显更差（对数秩检验 P=0.014，连续 Fisher 确切检验，P=0.008）。
我们的研究首次报告了 EMB 证实的心脏淀粉样变患者中存在较高的心肌内炎症发生率（48.1%），并表明在 AL 淀粉样变患者中，心肌内炎症与死亡率的增加显著相关。我们的数据具有直接的临床影响，因为人们可以假设，在心肌组织活检证实有炎症的患者中，额外的免疫调节/抗炎治疗方案可能对患有心脏 AL 淀粉样变的患者有益。
