具有自身免疫特征的间质性肺炎和组织学上的普通间质性肺炎采用抗纤维化与免疫抑制治疗。

Interstitial pneumonia with autoimmune features and histologic usual interstitial pneumonia treated with anti-fibrotic versus immunosuppressive therapy.

机构信息

Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.

Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan.

出版信息

Respir Investig. 2023 May;61(3):297-305. doi: 10.1016/j.resinv.2023.01.007. Epub 2023 Feb 28.

DOI:10.1016/j.resinv.2023.01.007

PMID:36863306

Abstract

BACKGROUND

Therapeutic strategies in patients with interstitial pneumonia with autoimmune features (IPAF) and histological usual interstitial pneumonia (UIP) pattern (IPAF-UIP) have not been thoroughly evaluated. We compared the therapeutic efficacy of anti-fibrotic therapy with that of immunosuppressive treatment for patients with IPAF-UIP.

METHODS

In this retrospective case series, we identified consecutive IPAF-UIP patients treated with anti-fibrotic therapy or immunosuppressive therapy. Clinical characteristics, one-year treatment response, acute exacerbation, and survival were studied. We performed a stratified analysis by the pathological presence or absence of inflammatory cell infiltration.

RESULTS

Twenty-seven patients with anti-fibrotic therapy and 29 with immunosuppressive treatment were included. There was a significant difference in one-year forced vital capacity (FVC) change between patients with anti-fibrotic treatment (4 in 27 improved, 12 stable, and 11 worsened) and those with immunosuppressive treatment (16 in 29 improved, eight stable, and five worsened) (p = 0.006). There was also a significant difference in one-year St George's Respiratory Questionnaire (SGRQ) change between patients with anti-fibrotic therapy (2 in 27 improved, ten stable, and 15 worsened) and those with immunosuppressive treatment (14 in 29 improved, 12 stable, and worsened) (p < 0.001). There was no significant difference in survival between the groups (p = 0.32). However, in the subgroup with histological inflammatory cell infiltration, survival was significantly better with immunosuppressive therapy (p = 0.02).

CONCLUSION

In IPAF-UIP, immunosuppressive therapy seemed to be superior to anti-fibrotic treatment in terms of therapeutic response, and provided better outcomes in the histological inflammatory subgroup. Further prospective studies are needed to clarify the therapeutic strategy in IPAF-UIP.

摘要

背景

具有自身免疫特征的间质性肺炎（IPAF）和组织学普通间质性肺炎（UIP）模式（IPAF-UIP）患者的治疗策略尚未得到彻底评估。我们比较了抗纤维化治疗与免疫抑制治疗对 IPAF-UIP 患者的疗效。

方法

在这项回顾性病例系列研究中，我们确定了接受抗纤维化治疗或免疫抑制治疗的连续 IPAF-UIP 患者。研究了临床特征、一年治疗反应、急性加重和生存情况。我们对存在或不存在炎症细胞浸润的病理进行了分层分析。

结果

27 例患者接受抗纤维化治疗，29 例患者接受免疫抑制治疗。抗纤维化治疗组（27 例中有 4 例改善，12 例稳定，11 例恶化）和免疫抑制治疗组（29 例中有 16 例改善，8 例稳定，5 例恶化）一年时用力肺活量（FVC）变化有显著差异（p=0.006）。抗纤维化治疗组（27 例中有 2 例改善，10 例稳定，15 例恶化）和免疫抑制治疗组（29 例中有 14 例改善，12 例稳定，5 例恶化）一年时圣乔治呼吸问卷（SGRQ）变化也有显著差异（p<0.001）。两组之间的生存率无显著差异（p=0.32）。然而，在组织学炎症细胞浸润亚组中，免疫抑制治疗的生存率显著更好（p=0.02）。