Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome
Pulmonary arterial hypertension associated with systemic sclerosis in patients with functional class II dyspnoea: mild symptoms but severe outcome
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DOI：
10.1093/rheumatology/kep449
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发表时间：
2010-05-01
期刊：
影响因子：
5.5
通讯作者：
Humbert, Marc
中科院分区：
文献类型：
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作者：
Hachulla, Eric;Launay, David;Humbert, Marc
客观的。描述纽约心脏协会 (NYHA) 功能分级 (FC) II 型呼吸困难患者诊断时的 SSc 相关肺动脉高压 (SSc-PAH) 病史。方法。收集并回顾性分析 77 名连续 SSc-PAH 患者诊断时的数据。结果。 12 名患者 (15.6%) 出现 PAH 和 NYHA FC II 呼吸困难。经过平均 44 个月的随访，12 名 PAH 患者中只有 4 名 FC II 保持稳定，而 8 名则恶化至 FC III 或 IV。观察期间死亡3例；两名来自 PAH，一名来自直肠癌。 FC II 患者诊断时的 1 年、2 年和 3 年生存率分别为 100%、91% 和 80%。结论。 NYHA FC II 诊断时大多数症状轻微的 SSc-PAH 患者病情严重，预后不良。
Objective. To describe the history of SSc-associated pulmonary arterial hypertension (SSc-PAH) in patients with New York Heart Association (NYHA) functional class (FC) II dyspnoea at diagnosis.Methods. Data at the time of diagnosis were collected and analysed retrospectively for 77 consecutive patients with SSc-PAH.Results. Twelve patients (15.6%) presented with PAH and NYHA FC II dyspnoea. After a mean follow-up of 44 months, only 4 out of the 12 PAH patients remained stable in FC II, while 8 showed worsening to FC III or IV. Three patients died during the observation period; two from PAH and one from rectal cancer. Survival in patients in FC II at diagnosis was 100, 91 and 80% at 1, 2 and 3 years, respectively.Conclusions. A majority of patients with mildly symptomatic SSc-PAH in NYHA FC II at diagnosis have a severe disease with poor prognosis.