Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan; Health Administration Center, Hamamatsu University School of Medicine, Hamamatsu, Japan.
Department of Diagnostic Radiology, Sakai City Medical Centre, Osaka, Japan.
Respir Med. 2020 Nov-Dec;174:106201. doi: 10.1016/j.rmed.2020.106201. Epub 2020 Oct 21.
Nonspecific interstitial pneumonia (NSIP) and organizing pneumonia (OP) are major subtypes of idiopathic interstitial pneumonias (IIPs) and closely related to connective tissue diseases (CTDs). "NSIP with OP overlap" is a controversial finding that has recently appeared in the criteria of interstitial pneumonia with autoimmune features (IPAF). However, details of this controversial entity are not well known.
To determine the frequency of "NSIP with OP overlap" pattern in IIPs and to identify differences from idiopathic NSIP (iNSIP).
In 524 patients with interstitial pneumonia from 39 institutes who underwent surgical lung biopsy, 444 were diagnosed as IIPs by a multidisciplinary discussion meeting via a cloud-based integrated database. Among these patients, 44 (9.9%) who had iNSIP and 21 (4.7%) with histopathologically-defined "NSIP with OP overlap" pattern (a pathological NSIP and OP pattern, but without a UIP pattern) were retrospectively studied.
Patients with "NSIP with OP overlap" pattern showed a significantly greater extent of consolidation (p < 0.001), more subpleural ground glass attenuation (p = 0.036), and more peripheral + bronchovascular distribution (p = 0.009) on high-resolution computed tomography than those with iNSIP. The incidences of newly-developed CTDs during follow-up was similar between the groups and polymyositis/dermatomyositis was the most frequent CTD in both groups. Nearly half of the patients fulfilled IPAF criteria, but no significant difference was found between iNSIP and "NSIP with OP overlap" pattern (47.7% vs. 42.9, p = 0.712). The incidence of acute exacerbation and the survival rates were similar between the groups.
The incidence of "NSIP with OP overlap" pattern is 4.7% in IIPs. The frequency of newly-developed CTDs during follow-up, mainly polymyositis/dermatomyositis, the frequency of acute exacerbation, and the survival rate in "NSIP with OP overlap" pattern are similar to those of iNSIP.
非特异性间质性肺炎(NSIP)和机化性肺炎(OP)是特发性间质性肺炎(IIP)的主要亚型,与结缔组织疾病(CTD)密切相关。“NSIP 与 OP 重叠”是一个有争议的发现,最近出现在具有自身免疫特征的间质性肺炎(IPAF)的标准中。然而,这个有争议的实体的细节尚不清楚。
确定 IIP 中“NSIP 与 OP 重叠”模式的频率,并确定其与特发性 NSIP(iNSIP)的差异。
在 39 家机构的 524 名接受外科肺活检的间质性肺炎患者中,通过基于云的综合数据库的多学科讨论会议,444 名患者被诊断为 IIP。在这些患者中,有 44 名(9.9%)患有 iNSIP,21 名(4.7%)患有组织病理学定义的“NSIP 与 OP 重叠”模式(一种病理 NSIP 和 OP 模式,但没有 UIP 模式),对这些患者进行回顾性研究。
“NSIP 与 OP 重叠”模式的患者在高分辨率计算机断层扫描上表现出明显更大程度的实变(p<0.001)、更多的胸膜下磨玻璃样衰减(p=0.036)和更多的外周+支气管血管分布(p=0.009),与 iNSIP 相比。在随访期间,新发 CTD 的发生率在两组之间相似,多发性肌炎/皮肌炎是两组中最常见的 CTD。几乎一半的患者符合 IPAF 标准,但 iNSIP 与“NSIP 与 OP 重叠”模式之间无显著差异(47.7%与 42.9%,p=0.712)。两组的急性加重发生率和生存率相似。
在 IIP 中,“NSIP 与 OP 重叠”模式的发生率为 4.7%。在随访期间新发 CTD 的频率,主要是多发性肌炎/皮肌炎、急性加重的频率以及“NSIP 与 OP 重叠”模式的生存率与 iNSIP 相似。