Introduction

Bronchiolitis obliterans syndrome (BOS) after allogeneic haemopoietic stem cell transplant (HSCT) is an under-recognised and difficult to treat disease. This occurs in the context of limited clinical research and inconsistent diagnostic criteria.

Method

Retrospective data was collected on 275 patients who underwent allogeneic HSCT at an Australian tertiary hospital between 2007 and 2017. The prevalence of BOS, defined by 2014 National Institute of Health criteria, as well as predictors for BOS and mortality were determined. Treatment outcomes, using serial spirometry, were compared between patients who received early versus late immunosuppression for BOS.

Results

The prevalence of BOS was 9.1%. Myeloablative conditioning (OR: 2.7, 95%CI: 1.13–6.50, p = 0.03) and extra-pulmonary chronic graft-versus-host disease (OR 2.62, 95% CI: 1.04–6.60, p = 0.04) were associated with BOS. There was reduced median survival in the BOS group compared with the non-BOS group, but this was not statistically significant (4.1years (IQR: 2.8, 6.8) versus 4.6years (IQR: 2.4, 7.8), respectively, p = 0.33). The vast majority (87.5%) of BOS patients failed to attain improvement in FEV1 at 12 months, regardless of treatment strategy. Patients who underwent a late immunosuppression strategy had worse mean FEV1 decline compared to those who received early immunosuppression (−36.3% versus −1.6%, respectively, p = 0.03).

Conclusion

BOS is a common and progressive disease following HSCT and is largely refractory to current treatment strategies. Compared to late immunosuppression, early augmentation of immunosuppression may slow lung function deterioration in the short term. However, further research is urgently needed to identify effective prevention and treatment strategies for BOS.

中文翻译：

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同种异体干细胞移植后闭塞性细支气管炎综合征的患病率、预测因素和治疗结果的更新

介绍

异基因造血干细胞移植（HSCT）后的闭塞性细支气管炎综合征（BOS）是一种未被充分认识且难以治疗的疾病。这是在临床研究有限和诊断标准不一致的情况下发生的。

方法

收集了 2007 年至 2017 年间在澳大利亚三级医院接受同种异体 HSCT 的 275 名患者的回顾性数据。确定了根据 2014 年美国国立卫生研究院标准定义的 BOS 患病率，以及 BOS 和死亡率的预测因素。使用系列肺活量测定法，对接受早期和晚期 BOS 免疫抑制的患者的治疗结果进行比较。

结果

BOS 的患病率为 9.1%。清髓性调节（OR：2.7，95% CI：1.13-6.50，p = 0.03）和肺外慢性移植物抗宿主病（OR 2.62，95% CI：1.04-6.60，p = 0.04）与 BOS 相关。与非 BOS 组相比，BOS 组的中位生存期有所缩短，但这并不具有统计学意义（分别为 4.1 年（IQR：2.8、6.8）与 4.6 年（IQR：2.4、7.8），p = 0.33） 。无论采用何种治疗策略，绝大多数 (87.5%) BOS 患者在 12 个月时 FEV1 均未能改善。与接受早期免疫抑制的患者相比，接受晚期免疫抑制策略的患者平均 FEV1 下降更严重（分别为 -36.3% 和 -1.6%，p = 0.03）。

结论

BOS 是 HSCT 后常见的进行性疾病，目前的治疗策略在很大程度上难以治愈。与晚期免疫抑制相比，早期增强免疫抑制可能会在短期内减缓肺功能恶化。然而，迫切需要进一步研究来确定 BOS 的有效预防和治疗策略。