Respiratory Franchise, Adelphi Real World, Adelphi Mill, Grimshaw Ln, Bollington, Macclesfield, SK10 5JB, UK.
Medical Affairs, Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson, Allschwil, Switzerland.
Ther Adv Respir Dis. 2024 Jan-Dec;18:17534666231218886. doi: 10.1177/17534666231218886.
Pulmonary arterial hypertension (PAH) is a life-threatening, progressive disease often diagnosed late in its course.
To present patient-reported data that were captured within a large, multinational, point-in-time survey of PAH-treating physicians and their patients to better understand the diagnostic journey.
Cross-sectional survey conducted in five European countries (EU5), Japan and the USA.
PAH-treating pulmonologists, cardiologists, rheumatologists or internists (USA only) completed a patient record form (PRF) for the next four consecutive adult PAH patients they saw; these patients filled in a patient self-completion (PSC) form on an anonymous, voluntary basis. Our report focuses on patient data; data are from PSC forms unless stated otherwise.
Physician-reported PRFs and self-completed PSC forms were obtained for 1152 and 572 patients, respectively. Patients' mean (SD) age was 59.1 (14.0) years, 55.6% were female, and 57.3% had idiopathic PAH. Patient-reported data showed an average delay of 17.0 months between symptom onset and PAH diagnosis. This is longer than physicians estimated (13.8 months): this disparity may be partly due to the time taken by patients to consult a physician about their symptoms [9.6 months overall, longest in the USA (15.3 months)]. Most patients (71.6%) initially consulted primary care physicians about their symptoms and 76.4% of patients were referred to a specialist. Misdiagnoses occurred in 40.9% of patients [most frequent in the USA (51.3%), least common in Japan (27.6%)] and they saw an average of 2.9 physicians overall (3.5 in EU5 2.0 in Japan/USA) before being diagnosed. Diagnosis was most often made by cardiologists (50.4%) or pulmonologists (49.3%).
Our data suggest that diagnostic delay in PAH results from patient- and physician-related factors, which differ across regions and include lack of awareness of PAH on both sides. Development of better screening strategies may help address this barrier to timely PAH diagnosis.
肺动脉高压（PAH）是一种危及生命的进行性疾病，通常在病程晚期才被诊断出来。
介绍在一项针对 PAH 治疗医生及其患者的大型、多国家、时点调查中收集的患者报告数据，以更好地了解诊断过程。
在五个欧洲国家（欧盟 5 国）、日本和美国进行的横断面调查。
PAH 治疗的肺科医生、心脏病专家、风湿病专家或内科医生（仅在美国）为他们接下来看到的连续 4 名成年 PAH 患者填写患者病历表（PRF）；这些患者在匿名自愿的基础上填写了一份患者自我完成（PSC）表格。我们的报告重点介绍患者数据；除非另有说明，否则数据来自 PSC 表格。
分别获得了医生报告的 PRF 和自我完成的 PSC 表格 1152 份和 572 份。患者的平均（SD）年龄为 59.1（14.0）岁，55.6%为女性，57.3%为特发性 PAH。患者报告的数据显示，从症状出现到 PAH 诊断的平均延迟为 17.0 个月。这比医生估计的时间要长（13.8 个月）：这种差异可能部分归因于患者向医生咨询症状所花费的时间[总体为 9.6 个月，在美国最长（15.3 个月）]。大多数患者（71.6%）最初向初级保健医生咨询他们的症状，76.4%的患者被转介给专科医生。有 40.9%的患者发生误诊[最常见于美国（51.3%），日本最少（27.6%）]，他们在被诊断之前平均看了 2.9 名医生（欧盟 5 国 3.5 名，日本/美国 2.0 名）。诊断最常由心脏病专家（50.4%）或肺科医生（49.3%）做出。
我们的数据表明，PAH 的诊断延迟是由患者和医生相关因素导致的，这些因素因地区而异，包括双方对 PAH 的认识不足。开发更好的筛查策略可能有助于解决及时诊断 PAH 的这一障碍。
