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

IF 5.2 2区 医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Georgia Trakada, Despina Fotiou, Anastasios Kallianos, Foteini Theodorakakou, Magdalini Migkou, Maria Gavriatopoulou, Nikolaos Kanellias, Panagiotis Malandrakis, Ioannis Ntanasis-Stathopoulos, Evangelos Eleutherakis-Papaiakovou, Ioanna Dialoupi, Evangelos Terpos, Meletios A Dimopoulos, Efstathios Kastritis
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引用次数: 0

Abstract

Background: Lung involvement in AL amyloidosis is not very common, but post-mortem data and retrospective studies suggest it is likely underrecognized.

Aim: To perform a comprehensive evaluation of lung function with pulmonary function tests (PFTs) in patients with newly diagnosed AL amyloidosis.

Methods: A prospective, non-interventional study of 139 consecutive patients with newly diagnosed AL amyloidosis.

Results: 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 <80% of predicted value, forced expiratory volume <80% of predicted value, and carbon monoxide diffusion capacity <70% were independently associated with poorer survival. Respiratory muscle strength (as assessed by maximal expiratory (Pe) and inspiratory (Pi) pressure) was affected in most patients (64% had Pi < 55% and 57% had Pe < 70% of predicted values). Pe% was an independent prognostic factor for survival (HR: 0.984 per 1% unit increase, p = 0.007).

Conclusions: 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.

肺功能检查显示全身性AL淀粉样变性患者未被识别的肺功能障碍,具有独立的预后意义。
背景:AL淀粉样变累及肺部并不常见,但尸检数据和回顾性研究表明,它可能未被充分认识。目的:应用肺功能试验(PFTs)对新诊断的AL淀粉样变性患者的肺功能进行综合评价。方法:对139例新诊断的AL淀粉样变患者进行前瞻性、非干预性研究。结果:PFTs显示68%的患者呼吸生理正常,9%为阻塞性,23%为限制性;后者与较差的生存相关(28.6 vs 76个月的阻塞性/正常生理,p = 0.002),并且在调整Mayo分期和异常胸部ct后仍然显著。强迫肺活量p = 0.007)。结论:pft评估的肺功能障碍在全身性AL淀粉样变性患者中很常见,但未被充分认识,具有重要的预后和潜在的治疗意义,与心功能障碍程度或胸部ct表现无关。
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来源期刊
Amyloid-Journal of Protein Folding Disorders
Amyloid-Journal of Protein Folding Disorders 生物-生化与分子生物学
CiteScore
10.60
自引率
10.90%
发文量
48
审稿时长
6-12 weeks
期刊介绍: Amyloid: the Journal of Protein Folding Disorders is dedicated to the study of all aspects of the protein groups and associated disorders that are classified as the amyloidoses as well as other disorders associated with abnormal protein folding. The journals major focus points are: etiology, pathogenesis, histopathology, chemical structure, nature of fibrillogenesis; whilst also publishing papers on the basic and chemical genetic aspects of many of these disorders. Amyloid is recognised as one of the leading publications on amyloid protein classifications and the associated disorders, as well as clinical studies on all aspects of amyloid related neurodegenerative diseases and major clinical studies on inherited amyloidosis, especially those related to transthyretin. The Journal also publishes book reviews, meeting reports, editorials, thesis abstracts, review articles and symposia in the various areas listed above.
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