Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Aichi, Japan.
Respir Investig. 2023 May;61(3):297-305. doi: 10.1016/j.resinv.2023.01.007. Epub 2023 Feb 28.
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.
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.
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).
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.
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。
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。
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）。
在 IPAF-UIP 中，免疫抑制治疗在治疗反应方面似乎优于抗纤维化治疗，并且在组织学炎症亚组中提供了更好的结果。需要进一步的前瞻性研究来阐明 IPAF-UIP 的治疗策略。
