Li Yi, Yi Qun
Department of Critical Care Medicine, West China Hospital of Sichuan University Chengdu 610041, China.
Department of Respiratory Medicine, West China Hospital of Sichuan University Chengdu 610041, China.
Int J Clin Exp Med. 2015 Sep 15;8(9):16850-3. eCollection 2015.
Pulmonary arterial hypertension (PAH) and ANCA-associated vasculitis (AAV) are both rare and complex diseases with poor prognosis especially when misdiagnosis. We report a rare case of a young woman presented with idiopathic pulmonary arterial hypertension (IPAH), but who was later found to have PAH associated with AAV. This case reminds us the very importance of differential diagnosis to PAH patients and the identification of rare cause likes ANCA-associated vasculitis. To our knowledge, this patient represents the first published case of misdiagnosis of PAH secondary to ANCA-associated vasculitis.
肺动脉高压（PAH）和抗中性粒细胞胞浆抗体相关性血管炎（AAV）均为罕见且复杂的疾病，预后较差，尤其是在误诊的情况下。我们报告了一例罕见病例，一名年轻女性最初表现为特发性肺动脉高压（IPAH），但后来发现其PAH与AAV相关。该病例提醒我们对PAH患者进行鉴别诊断以及识别如ANCA相关性血管炎等罕见病因的重要性。据我们所知，该患者是首例发表的因ANCA相关性血管炎继发PAH误诊的病例。
