S50 Hypersensitivity pneumonitis complicated by pulmonary hypertension; patient characteristics and response to targeted therapy: Abstract S50 Table 1.
S50 Hypersensitivity pneumonitis complicated by pulmonary hypertension; patient characteristics and response to targeted therapy: Abstract S50 Table 1.
复制标题
S50 过敏性肺炎并发肺动脉高压；
DOI：
10.1136/thoraxjnl-2013-204457.57
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发表时间：
2013
期刊：
影响因子：
10
通讯作者：
Garfield B
中科院分区：
文献类型：
--
作者：
Garfield B
背景过敏性肺炎（HP）是由反复接触致敏抗原（通常是有机颗粒）引起的。它可以是急性、亚急性或慢性的 (1)。很少有文献描述肺动脉高压 (PH) 与 HP 的关系 (2)。我们的目的是总结三级转诊中心 HP 和 PH 患者的临床特征和结果，包括对靶向治疗的反应。方法通过中央数据库确定 1992 年至 2008 年间诊断的病例。 PH定义为右心导管平均肺动脉压≥25mmHg或超声心动图右心室收缩压≥50mmHg。通过案例记录和电子记录审查收集数据。使用 Graphpad 棱镜进行分析。结果 确定了 19 名连续患有 PH 和 HP 的患者。 HRCT 显示纤维化证明 15 人患有慢性 HP，而 4 人患有亚急性疾病。表 1 列出了人口统计学和基线结果。14 名患者发现右心室 (RV) 功能障碍。 10 人接受了靶向治疗（西地那非+/-内皮素受体拮抗剂），其中 7 人有右心室功能障碍的证据。在西地那非测量前和第一次测量后，中位 BNP 从 80 pmol/L 下降至 27 pmol/L，但未能达到统计学显着性 (p = 0.11)。在中位 4 个月的超声心动图随访中，6 名患者中的 2 名患者的右心室功能得到改善。治疗期间 RVSP、TR 速度或肺加速时间没有一致的变化。 12 名患者在诊断 PH 后平均 8 个月 (0.2 - 46) 内死亡。虽然未达到统计学显着性，但幸存者的中位动脉 pO2 较高（7.25 与 5.85 kPa：p = 0.11）。结论 慢性和亚急性 HP 可能并发 PH。尽管进行了靶向治疗，该组的死亡率仍然极高。死亡者的 PO2 普遍较低。 HP 患者的 PH 诊断很可能是临终事件。需要进一步的工作来了解哪些患者可能对先进疗法有反应。参考文献A Wells 和 N Hirani (2008) 间质性肺疾病指南。胸部。 63v1–v58D S Koschelet al.(2012)肺。 190（3）：295–302
BackgroundHypersensitivity pneumonitis (HP) results from repeated exposure to a sensitizing antigen, normally an organic particle. It can be acute, sub-acute or chronic (1). There is very little literature describing the association of pulmonary hypertension (PH) with HP (2). We aimed to summarize the clinical characteristics and outcomes including responses to targeted therapy in patients with HP and PH in a tertiary referral centre.MethodsCases diagnosed between 1992 and 2008 were identified through a central database. PH was defined as mean pulmonary artery pressure ≥25mmHg on right heart catheter or right ventricular systolic pressure of ≥50 mmHg on echocardiogram. Data was collected through case note and electronic record review. Analysis was performed using Graphpad prism.ResultsNineteen consecutive patients with PH and HP were identified. Fifteen had chronic HP as evidenced by fibrosis on HRCT, whilst 4 had sub-acute disease. Demographic and baseline findings are listed in table 1. Right ventricular (RV) dysfunction was found in 14 patients. Ten were treated with targeted therapy (sildenafil +/- endothelin receptor antagonist), of these 7 had evidence of RV dysfunction. Median BNP fell between pre and first post sildenafil measurement from 80 to 27 pmol/L but failed to reach statistical significance (p = 0.11). RV function improved in 2 of 6 patients with available follow up echocardiogram at a median of 4 months. There was no consistent change in RVSP, TR velocity or pulmonary acceleration time with treatment. Twelve patients died, a median of 8 months (0.2 - 46) from diagnosis of PH. Although not reaching statistical significance, the median arterial pO2was higher in survivors (7.25 versus 5.85 kPa: p = 0.11).ConclusionChronic and sub-acute HP can be complicated by PH. Mortality in this group was extremely high despite targeted therapy. PO2was generally lower in those who died. The diagnosis of PH in HP patients may well be a pre-terminal event. Further work is needed to understand which patients may respond to advanced therapies.ReferencesA Wells and N Hirani (2008) Interstitial Lung Disease Guidelines.Thorax. 63v1–v58D S Koschelet al.(2012)Lung. 190(3):295–302