Pulmonary function tests reveal unrecognised lung dysfunction and have independent prognostic significance in patients with systemic AL amyloidosis

Lung involvement in AL amyloidosis is not very common, but post-mortem data and retrospective studies suggest it is likely underrecognized. To perform a comprehensive evaluation of lung function with pulmonary function tests (PFTs) in patients with newly diagnosed AL amyloidosis. A prospective, non-interventional study of 139 consecutive patients with newly diagnosed AL amyloidosis. PFTs indicated normal breathing physiology in 68% of patients, obstructive in 9% and restrictive in 23%; the latter was associated with worse survival (28.6 vs 76 months for obstructive/normal physiology, p = 0.002) and remained significant after adjustment for Mayo stage and abnormal chest-CT. Forced vital capacity p = 0.007). Pulmonary dysfunction, as assessed with PFTs, is common and underrecognized in patients with systemic AL amyloidosis, with significant prognostic and potentially therapeutic implications, independent of the degree of cardiac dysfunction or chest-CT findings.AL型淀粉样变性（AL amyloidosis）的肺部受累并不多见，但尸检数据与回顾性研究表明，该病症的肺部受累很可能存在识别不足的情况。本研究旨在通过肺功能检查（pulmonary function tests, PFTs）对新确诊的AL型淀粉样变性患者开展全面的肺功能评估。本研究为一项针对139例连续入组的新确诊AL型淀粉样变性患者的前瞻性非干预性研究。肺功能检查结果显示，68%的患者呼吸生理功能正常，9%存在阻塞性通气功能障碍，23%存在限制性通气功能障碍；其中限制性通气功能障碍与较差的生存率相关（阻塞性/通气功能正常患者的生存期为76个月，该类患者仅为28.6个月，p=0.002），且在校正Mayo分期与胸部CT异常结果后，该关联仍具有统计学意义。用力肺活量相关分析的p值为0.007。通过肺功能检查评估的肺功能异常，在系统性AL型淀粉样变性患者中十分常见且常被漏诊，其具有重要的预后价值及潜在的临床治疗指导意义，且该相关性不受心脏功能障碍程度或胸部CT检查结果的影响。