显微镜下多血管炎中间质性肺病复发的不良预后因素：日本多中心REVEAL队列研究
Poor prognostic factors for relapse of interstitial lung disease in microscopic polyangiitis: the Japanese multicentre REVEAL cohort study.
作者信息
Matsuda Shogo, Kotani Takuya, Okazaki Ayana, Nishioka Daisuke, Masuda Yuichi, Shiomi Mayu, Watanabe Ryu, Taniguchi Tomoki, Manabe Atsushi, Kadoba Keiichiro, Yoshida Tsuneyasu, Hiwa Ryosuke, Yamamoto Wataru, Hashimoto Motomu, Takeuchi Tohru
机构信息
Department of Internal Medicine IV, Division of Rheumatology, Osaka Medical and Pharmaceutical University, Daigaku-Machi 2-7, Takatsuki, Osaka, 569-8686, Japan.
Department of Medical Statistics, Research & Development Center, Osaka Medical and Pharmaceutical University, Osaka, Japan.
出版信息
Arthritis Res Ther. 2024 Dec 19;26(1):221. doi: 10.1186/s13075-024-03453-z.
BACKGROUND
This study investigated poor prognostic factors for the relapse of interstitial lung disease (ILD) in patients with microscopic polyangiitis (MPA) after remission induction therapy.
METHODS
We enrolled patients diagnosed with MPA complicated by ILD according to the Chapel Hill Consensus definition from 2001 to 2023 in multiple institutions in the REVEAL cohort. All patients who were treated with immunosuppressive therapy were followed up, and those who relapsed with ILD were extracted in this study. We explored the risk factors for predicting ILD relapse in patients with MPA-ILD by comparing the demographic, clinical, laboratory, and radiological findings and treatments between the relapsed and non-relapsed groups on admission.
RESULTS
Of 243 patients with MPA, 134 (55.1%) with MPA-ILD were enrolled. Among them, 28 (20.9%) relapsed during a mean follow-up of 4.2 years. The initial serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) levels and the prevalence of usual interstitial pneumonia (UIP) pattern were significantly higher in the relapsed group. The biomarkers were also risk factors for relapse in multivariate Cox regression analysis. The best cut-off values of KL-6, SP-D for predicting ILD relapse were 430 U/mL and 89.5 ng/mL, respectively. We created prediction models based on the best cut-off values for KL-6, SP-D, and the presence of the UIP pattern (KSU model). The 10-year relapse rate was significantly different among patients with MPA-ILD stratified by the number of risk factors based on the KSU model. A higher relapse rate was associated with higher all-cause mortality.
CONCLUSIONS
The initial serum high KL-6 and SP-D levels and the prevalence of the UIP pattern were associated with ILD relapse in patients with MPA-ILD. Our multicentre cohort study indicated that the KSU model, which consists of KL-6 ≥ 430 U/mL, SP-D ≥ 89.5 ng/mL, and the presence of the UIP pattern, is a useful predictor of ILD relapse in patients with MPA after immunosuppressive therapy.
背景
本研究调查了显微镜下多血管炎（MPA）患者在诱导缓解治疗后间质性肺疾病（ILD）复发的不良预后因素。
方法
我们根据2001年至2023年的查珀尔希尔共识定义，在REVEAL队列的多个机构中纳入了诊断为MPA合并ILD的患者。对所有接受免疫抑制治疗的患者进行随访，并在本研究中提取那些ILD复发的患者。通过比较复发组和未复发组入院时的人口统计学、临床、实验室和影像学检查结果及治疗情况，我们探索了预测MPA-ILD患者ILD复发的危险因素。
结果
在243例MPA患者中，134例（55.1%）为MPA-ILD患者。其中，28例（20.9%）在平均4.2年的随访期间复发。复发组的初始血清克雷伯氏肺表面活性物质相关蛋白-6（KL-6）和表面活性蛋白-D（SP-D）水平以及普通间质性肺炎（UIP）模式的患病率显著更高。在多变量Cox回归分析中，这些生物标志物也是复发的危险因素。预测ILD复发的KL-6、SP-D的最佳截断值分别为430 U/mL和89.5 ng/mL。我们基于KL-6、SP-D的最佳截断值以及UIP模式的存在创建了预测模型（KSU模型）。根据KSU模型，按危险因素数量分层的MPA-ILD患者的10年复发率有显著差异。较高的复发率与较高的全因死亡率相关。
结论
初始血清高KL-6和SP-D水平以及UIP模式的患病率与MPA-ILD患者的ILD复发相关。我们的多中心队列研究表明，由KL-6≥430 U/mL、SP-D≥89.5 ng/mL以及UIP模式的存在组成的KSU模型是免疫抑制治疗后MPA患者ILD复发的有用预测指标。
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