Abstract：
BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has features of connective tissue disease-associated interstitial lung disease (CTD-ILD), but without meeting criteria for a specific CTD. We compared baseline characteristics, survival, and response to treatment of IPAF to both CTD-ILD and unclassifiable ILD.
METHODS: Measurements were extracted from a prospective registry. Baseline features and survival were compared in IPAF against both CTD-ILD and unclassifiable ILD. Linear trajectory of lung function decline (%-predicted forced vital capacity [FVC%] and diffusion capacity of the lung for carbon monoxide [DLCO%]) before and after initiation of mycophenolate or azathioprine were compared in IPAF against both CTD-ILD and unclassifiable ILD using linear mixed models.
RESULTS: Compared to CTD-ILD (n = 1240), patients with IPAF (n = 128) were older, more frequently male, and had greater smoking history. Compared to unclassifiable ILD (n = 665), patients with IPAF were younger, more frequently female, and had worse baseline lung function. IPAF had higher mortality compared to CTD-ILD and similar risk of mortality compared to unclassifiable ILD. Mycophenolate initiation was associated with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% in patients with IPAF, and azathioprine initiation with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% decline in IPAF and DLCO% decline in CTD-ILD.
CONCLUSIONS: Patients with IPAF had worse survival compared to those with CTD-ILD and similar mortality to unclassifiable ILD, with treatment being associated with stabilization in lung function in all three ILDs. It is uncertain whether IPAF should be considered a distinct ILD diagnostic subgroup.
METHODS: Measurements were extracted from a prospective registry. Baseline features and survival were compared in IPAF against both CTD-ILD and unclassifiable ILD. Linear trajectory of lung function decline (%-predicted forced vital capacity [FVC%] and diffusion capacity of the lung for carbon monoxide [DLCO%]) before and after initiation of mycophenolate or azathioprine were compared in IPAF against both CTD-ILD and unclassifiable ILD using linear mixed models.
RESULTS: Compared to CTD-ILD (n = 1240), patients with IPAF (n = 128) were older, more frequently male, and had greater smoking history. Compared to unclassifiable ILD (n = 665), patients with IPAF were younger, more frequently female, and had worse baseline lung function. IPAF had higher mortality compared to CTD-ILD and similar risk of mortality compared to unclassifiable ILD. Mycophenolate initiation was associated with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% in patients with IPAF, and azathioprine initiation with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% decline in IPAF and DLCO% decline in CTD-ILD.
CONCLUSIONS: Patients with IPAF had worse survival compared to those with CTD-ILD and similar mortality to unclassifiable ILD, with treatment being associated with stabilization in lung function in all three ILDs. It is uncertain whether IPAF should be considered a distinct ILD diagnostic subgroup.
摘要：
背景：具有自身免疫特征的间质性肺炎（IPAF）具有结缔组织疾病相关性间质性肺病（CTD-ILD）的特征，但不符合特定CTD的标准。我们比较了基线特征，生存,以及IPAF治疗对CTD-ILD和不可分类ILD的反应。
方法：从前瞻性注册中提取测量值。比较了IPAF与CTD-ILD和不可分类ILD的基线特征和生存率。在IPAF中比较了霉酚酸酯或硫唑嘌呤开始之前和之后的肺功能下降的线性轨迹（％预测的强迫肺活量[FVC％]和肺对一氧化碳的扩散能力[DLCO％]）与CTD-ILD和使用线性混合模型的不可分类ILD。
结果：与CTD-ILD（n=1240）相比，IPAF患者（n=128）年龄较大，更常见的是男性，有较多的吸烟史。与不可分类的ILD(n=665)相比，IPAF患者更年轻，更常见的是女性，基线肺功能较差。与CTD-ILD相比，IPAF的死亡率更高，与未分类的ILD相比，死亡率风险相似。霉酚酸酯的启动与所有ILD亚型的FVC％和DLCO％的稳定相关，IPAF患者的FVC％除外。除IPAF的FVC％下降和CTD-ILD的DLCO％下降外，所有ILD亚型的硫唑嘌呤起始和FVC％和DLCO％稳定。
结论：与CTD-ILD患者相比，IPAF患者的生存率更差，死亡率与无法分类的ILD相似。治疗与所有三种ILD的肺功能稳定相关。不确定是否应将IPAF视为不同的ILD诊断亚组。
方法：从前瞻性注册中提取测量值。比较了IPAF与CTD-ILD和不可分类ILD的基线特征和生存率。在IPAF中比较了霉酚酸酯或硫唑嘌呤开始之前和之后的肺功能下降的线性轨迹（％预测的强迫肺活量[FVC％]和肺对一氧化碳的扩散能力[DLCO％]）与CTD-ILD和使用线性混合模型的不可分类ILD。
结果：与CTD-ILD（n=1240）相比，IPAF患者（n=128）年龄较大，更常见的是男性，有较多的吸烟史。与不可分类的ILD(n=665)相比，IPAF患者更年轻，更常见的是女性，基线肺功能较差。与CTD-ILD相比，IPAF的死亡率更高，与未分类的ILD相比，死亡率风险相似。霉酚酸酯的启动与所有ILD亚型的FVC％和DLCO％的稳定相关，IPAF患者的FVC％除外。除IPAF的FVC％下降和CTD-ILD的DLCO％下降外，所有ILD亚型的硫唑嘌呤起始和FVC％和DLCO％稳定。
结论：与CTD-ILD患者相比，IPAF患者的生存率更差，死亡率与无法分类的ILD相似。治疗与所有三种ILD的肺功能稳定相关。不确定是否应将IPAF视为不同的ILD诊断亚组。
