Background

Interstitial lung disease (ILD) is associated with high morbidity and mortality in patients with connective tissue disease (CTD). Because some patients with CTD overlap present with ILD first, with CTD diagnosed later, specific radiologic signs are needed to help differentiate each CTD or CTD-ILD from idiopathic ILD.

Objectives

To determine whether specific CT findings can help differentiate CTD as rheumatoid arthritis (RA), systemic sclerosis (SSc), or polymyositis/dermatomyositis (PM/DM).

Methods

We analyzed 143 consecutive ILD patients with RA, SSc, or PM/DM. We assessed diagnostic accuracy of CT findings of CTD-ILD, CT pattern, and signs including “anterior upper lobe honeycomb-like lesion” and “low attenuation area (LAA) within an interstitial abnormality” for each CTD-ILD. Prognostic predictors were determined using Cox regression models.

Results

Subjects were 78 patients with RA-ILD, 38 with SSc-ILD, 24 with PM/DM-ILD, and 3 with overlapping CTD-ILD. High frequency of anterior upper lobe honeycomb-like lesion suggests that CTD-ILD is due to RA-ILD (22%) rather than SSc-ILD (8%) or PM/DM-ILD (8%), whereas LAA within an interstitial abnormality suggests that CTD-ILD is due to SSc-ILD (26%) rather than RA-ILD (4%) or PM/DM-ILD (0%). Multivariate analysis showed that while not associated with survival, current or ex-smoker, honeycombing, and acute exacerbation were negative prognostic factors of mortality.

Conclusions

The tendency is high for RA-ILD, in which anterior upper lobe honeycomb-like lesion is a specific feature, to show UIP or NSIP/UIP pattern, combined emphysema, and honeycombing; SSc-ILD to show NSIP pattern and LAA within an interstitial abnormality; and PM/DM-ILD to show NSIP pattern and non-honeycombing.

中文翻译：

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前上叶蜂窝状病变在结缔组织病相关的间质性肺病中的潜在用途。

背景

间质性肺疾病（ILD）与结缔组织病（CTD）患者的高发病率和死亡率相关。由于一些CTD重叠的患者首先出现ILD，后来被诊断出CTD，因此需要特殊的放射学体征来帮助区分每个CTD或CTD-ILD与特发性ILD。

目标

为了确定特定的CT表现是否有助于将CTD区分为类风湿性关节炎（RA），系统性硬化症（SSc）或多发性肌炎/皮肌炎（PM / DM）。

方法

我们分析了143例连续的ILD患者，患有RA，SSc或PM / DM。我们评估了每种CTD-ILD的CT征象，CT模式和体征包括“前上叶蜂窝状病变”和“间质异常内的低衰减区（LAA）”的体征的诊断准确性。使用Cox回归模型确定预后指标。

结果

受试者为78例RA-ILD，38例SSc-ILD，24例PM / DM-ILD和3例重叠CTD-ILD。前上叶蜂窝状病变的高频率提示CTD-ILD是由RA-ILD（22％）而非SSc-ILD（8％）或PM / DM-ILD（8％）引起的，而LAA位于间隙异常提示CTD-ILD是由SSc-ILD（26％）而非RA-ILD（4％）或PM / DM-ILD（0％）引起的。多变量分析表明，尽管与生存无关，但当前或以前吸烟者，蜂窝状和急性加重是死亡率的阴性预后因素。

结论

RA-ILD的趋势很高，其中前叶上样蜂窝状病变是特定特征，表现出UIP或NSIP / UIP模式，合并的肺气肿和蜂窝状。SSc-ILD在间质异常内显示NSIP模式和LAA；和PM / DM-ILD显示NSIP模式和非蜂巢状。