Significance of connective tissue disease features in idiopathic interstitial pneumonia
Significance of connective tissue disease features in idiopathic interstitial pneumonia
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DOI：
10.1183/09031936.00174910
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发表时间：
2012-03-01
影响因子：
24.3
通讯作者：
Wells, A. U.
中科院分区：
文献类型：
--
作者：
Corte, T. J.;Copley, S. J.;Wells, A. U.
在特发性间质性肺炎(IIP)中，在缺乏特定结缔组织病(CTD)诊断的情况下，结缔组织病(CTD)特征的意义尚不清楚。我们研究了未分化CTD(UCTD)在经活检证实的IIP患者中的临床和预后价值。IIP患者接受外科肺活检(1979-2005)(非特异性间质性肺炎(NSIP)，n=45；特发性肺纤维化，n=56)。当血清自身抗体存在且症状或体征提示CTD时，UCTD被认为存在。评价UCTD与NSIP组织学之间的关系。使用先验变量构建了最佳预测NSIP组织学的临床算法。对UCTD和该算法的预后价值进行了评估。14例(31%)NSIP和7例(13%)IPF患者存在UCTD。UCTD与生存福利无关。预测非胰岛素依赖型肺炎的算法(OR10.4，95%可信区间3.21-33.67；p<；0.0001)包括缺乏典型的特发性肺间质纤维化的高分辨率CT特征和1)符合标准的人口统计学特征(50岁的女性)或2)雷诺现象。在IPF的HRCT扫描不典型的患者中，该算法预测了独立于IIP严重程度的生存改善(风险比0.35，95%可信区间0.14-0.85；p=0.02)。UCTD与NSIP组织学相关。然而，UCTD在IIP患者中的诊断和预后意义仍不清楚。
In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP.IIP patients undergoing surgical lung biopsy (1979-2005) were studied (nonspecific interstitial pneumonia (NSIP), n=45; idiopathic pulmonary fibrosis, n=56). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated.UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p < 0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged < 50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p=0.02) independent of IIP severity.UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear.