Department of Respiratory Medicine and Lung Transplantation, Aix Marseille Univ, APHM, North Hospital, Marseille, France.
Center for Cardiovascular and Nutrition Research, Aix Marseille Univ, INSERM 1263, INRA 1260, Marseille, France.
BMC Pulm Med. 2024 Oct 28;24(1):537. doi: 10.1186/s12890-024-03361-7.
Systemic sclerosis-related interstitial lung disease (SSc-ILD) represents a significant cause of morbidity and mortality in Systemic Sclerosis (SSc). Mycophenolate mofetil (MMF) is currently the first line treatment for SSc-ILD. There is no recommendation on the dosage of mycophenolic acid (MPA) blood concentrations, so we aimed to study the correlation between MPA exposure and respiratory outcomes in this population.
We conducted a retrospective cohort study of SSc-ILD patients treated with MMF in our center. According to our policy, a complete patient evaluation was performed approximately one year after MMF initiation, during which the mycophenolic acid (MPA) residual rate (RR) was measured. We analyzed the association between RR and changes in forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO) over time.
Forty-three SSc-ILD patients were included. Patients with higher RR levels (≥ 1.5 mg/L) had a significantly better FVC evolution with a higher proportion of stabilization and lower proportion of FVC decrease (p = 0.024). RR above 1.5 mg/L was a predictive factor of reduced FVC decline compared with lower RR levels adjusting for MMF dose and duration of MMF exposure (p = 0.008). There was no difference regarding DLCO outcome.
Our study suggests that optimal MPA exposure, as indicated by RR levels, may better protect against FVC decline in SSc-ILD patients treated with MMF. Routine monitoring of MPA exposure could be beneficial in optimizing treatment outcomes. Prospective, multicenter studies are needed to further explore the relationship between MPA exposure and clinical outcomes in SSc-ILD.
系统性硬化症相关间质性肺病（SSc-ILD）是系统性硬化症（SSc）患者发病率和死亡率的重要原因。霉酚酸酯（MMF）目前是 SSc-ILD 的一线治疗药物。目前尚无关于霉酚酸（MPA）血药浓度的剂量推荐，因此我们旨在研究该人群中 MPA 暴露与呼吸结局之间的相关性。
我们对在我们中心接受 MMF 治疗的 SSc-ILD 患者进行了回顾性队列研究。根据我们的政策，在 MMF 启动后大约一年进行一次完整的患者评估，在此期间测量了霉酚酸（MPA）残留率（RR）。我们分析了 RR 与用力肺活量（FVC）和一氧化碳弥散量（DLCO）随时间的变化之间的关系。
共纳入 43 例 SSc-ILD 患者。RR 水平较高（≥1.5mg/L）的患者 FVC 演变明显更好，稳定比例较高，FVC 下降比例较低（p=0.024）。RR 高于 1.5mg/L 是与 RR 水平较低相比降低 FVC 下降的预测因子，调整 MMF 剂量和 MMF 暴露持续时间（p=0.008）。DLCO 结果无差异。
我们的研究表明，作为 RR 水平的 MPA 最佳暴露可能更好地保护 SSc-ILD 患者的 FVC 下降。常规监测 MPA 暴露可能有助于优化治疗结果。需要进行前瞻性、多中心研究，以进一步探讨 SSc-ILD 患者 MPA 暴露与临床结局之间的关系。
