Natural history of indolent-anti-synthetase syndrome-associated interstitial lung disease

IF 2.4 Q2 RESPIRATORY SYSTEM
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引用次数: 0

Abstract

Background

Anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD) may occur without myositis. Although a recent Japanese guide proposed a watch-and-wait approach for chronic ASS-ILD without obvious progression, the natural history of this subgroup and the appropriateness of the watch-and-wait approach remain unclear. We aimed to describe the natural history of ASS-ILD, that is sufficiently indolent to be a candidate for the watch-and-wait approach.

Methods

Among consecutive patients with ASS-ILD, we retrospectively identified those without myositis, acute/subacute onset, and significant lung function impairment, which qualified them as indolent-ASS-ILD cases, and described their natural course. Additionally, we evaluated the risk factors for fibrosis progression on computed tomography (CT) using the Cox proportional hazards model.

Results

Among 80 patients with ASS-ILD, we identified 33 with indolent-ASS-ILD, all of whom were initially followed up with a watch-and-wait approach. Among 30 patients with sufficient follow-up data, 27 (90%) showed a stable course without treatment over 24 months. Subsequently, four patients experienced ≥10% relative forced vital capacity (FVC) decline without treatment during a median follow-up duration of 81 months. Seven patients showed fibrosis progression with >10% increase in the total lung area on CT. Higher levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) were associated with fibrosis progression on CT.

Conclusion

Most patients with indolent-ASS-ILD did not experience ≥10% relative FVC decline over five years without treatment. However, fibrosis progression on CT, which seemed to precede significant FVC decline, occurred more frequently, especially in patients with higher KL-6 and SP-D levels.

吲哚抗合成酶综合征相关间质性肺病的自然病史。
背景:抗合成酶综合征相关间质性肺病(ASS-ILD抗合成酶综合征相关间质性肺病(ASS-ILD)可能在没有肌炎的情况下发生。尽管日本最近的一份指南建议对无明显进展的慢性 ASS-ILD 采取观察和等待的方法,但这一亚群的自然病史以及观察和等待方法的适当性仍不清楚。我们的目的是描述ASS-ILD的自然病史:在连续的 ASS-ILD 患者中,我们回顾性地确定了那些没有肌炎、急性/亚急性起病和明显肺功能损害的患者,这些患者符合惰性 ASS-ILD 病例的条件,并描述了他们的自然病程。此外,我们还使用 Cox 比例危险模型评估了计算机断层扫描(CT)纤维化进展的风险因素:在80名ASS-ILD患者中,我们发现了33名吲哚-ASS-ILD患者,所有这些患者最初都接受了观察和等待法随访。在 30 名有足够随访数据的患者中,27 人(90%)在 24 个月内病情稳定,无需治疗。随后,在中位 81 个月的随访期间,4 名患者在未接受治疗的情况下,相对用力肺活量(FVC)下降≥10%。七名患者出现纤维化进展,CT显示肺总面积增加>10%。CT 上较高水平的 Krebs von den Lungen-6 (KL-6) 和表面活性蛋白-D (SP-D) 与纤维化进展有关:结论:大多数吲哚-ASS-ILD患者在未经治疗的情况下,五年内相对肺活量下降不超过10%。结论:大多数吲哚-ASS-ILD 患者在未经治疗的情况下,其肺活量在五年内不会相对下降≥10%。然而,CT 上的纤维化进展似乎先于肺活量的显著下降,这在 KL-6 和 SP-D 水平较高的患者中发生得更为频繁。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Respiratory investigation
Respiratory investigation RESPIRATORY SYSTEM-
CiteScore
4.90
自引率
6.50%
发文量
114
审稿时长
64 days
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