2nd Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan.
Respir Med. 2010 Oct;104(10):1527-34. doi: 10.1016/j.rmed.2010.04.022. Epub 2010 May 18.
Although idiopathic nonspecific interstitial pneumonia (NSIP) was initially identified as a provisional diagnosis, the 2008 American Thoracic Society Project concluded that idiopathic NSIP is a distinct form of idiopathic interstitial pneumonia. However, an association between idiopathic NSIP and autoimmune diseases still attracts interest. In this context, a recent study proposed an intriguing concept that idiopathic NSIP is the pulmonary manifestation of undifferentiated connective tissue disease (UCTD). However, this has not been confirmed in a large number of patients with idiopathic NSIP. The present study was conducted to investigate the proportion and characteristics of patients with idiopathic NSIP who meet the criteria for UCTD.
We reviewed 47 consecutive patients with idiopathic NSIP and examined whether they met prespecified criteria for UCTD. Furthermore, we compared the clinical characteristics between patients fulfilling the UCTD criteria (UCTD-NSIP) and those not meeting them (Non-UCTD-NSIP).
Of 47 patients with idiopathic NSIP, 22 (47%) met the UCTD criteria. Common symptoms associated with connective tissue diseases (CTDs) were skin change (50%) and Raynaud's phenomenon (41%) in UCTD-NSIP. UCTD-NSIP showed a female predominance and significantly higher percentages of lymphocytes with a lower CD4/CD8 ratio in bronchoalveolar lavage than Non-UCTD-NSIP. Interestingly, UCTD-NSIP had a significantly better survival than Non-UCTD-NSIP.
Idiopathic NSIP included subjects who fulfilled the UCTD criteria, and these subjects had different clinical characteristics with significantly better outcome than those who did not meet the criteria. These data suggest that a part, but not all, of patients with idiopathic NSIP show CTD-like features with a distinct prognosis.
虽然特发性非特异性间质性肺炎（NSIP）最初被确定为一种临时性诊断，但 2008 年美国胸科学会的项目得出结论，特发性 NSIP 是一种特发性间质性肺炎的独特形式。然而，特发性 NSIP 与自身免疫性疾病之间的关联仍然引起关注。在这种情况下，最近的一项研究提出了一个有趣的概念，即特发性 NSIP 是未分化结缔组织病（UCTD）的肺部表现。然而，这在大量特发性 NSIP 患者中尚未得到证实。本研究旨在调查符合 UCTD 标准的特发性 NSIP 患者的比例和特征。
我们回顾了 47 例连续的特发性 NSIP 患者，并检查了他们是否符合 UCTD 的预定义标准。此外，我们比较了符合 UCTD 标准的患者（UCTD-NSIP）和不符合标准的患者（非 UCTD-NSIP）的临床特征。
在 47 例特发性 NSIP 患者中，有 22 例（47%）符合 UCTD 标准。与结缔组织疾病（CTD）相关的常见症状是皮肤改变（50%）和雷诺现象（41%）。UCTD-NSIP 以女性为主，支气管肺泡灌洗液中的淋巴细胞比例较高，CD4/CD8 比值较低，而非 UCTD-NSIP 则相反。有趣的是，UCTD-NSIP 的生存率明显高于非 UCTD-NSIP。
特发性 NSIP 包括符合 UCTD 标准的患者，这些患者的临床特征不同，预后明显优于不符合标准的患者。这些数据表明，一部分但不是全部的特发性 NSIP 患者具有 CTD 样特征，并具有独特的预后。
