Pulmonary Capillary Hemangiomatosis in a Patient with Polymyositis: A Case Report and Literature Review

一個多發性肌炎患者的肺微血管瘤病變：病例報告及文獻回顧

陳建維(Chian-Wei Chen) ； 黃堂修(Tang-Hsiu Huang) ； 張漢煜(Han-Yu Chang) ； 陳昌文(Chang-Wen Chen)

《胸腔醫學》 33卷5期 (2018/10) Pp. 190-196

摘要

Pulmonary capillary hemangiomatosis (PCH) is characterized by abnormally proliferating capillaries that invade the pulmonary interstitium and alveolar septae, and occlude the normal pulmonary vasculature. It is a rare cause of pulmonary hypertension, with an unknown pathogenesis and a very poor prognosis. The clinical presentation of PCH is nonspecific, and radiological images often show diffuse centrilobular ground-glass opacities. Pathology examination is required for diagnosis. It is important to differentiate whether the pulmonary hypertension is caused by PCH, because in these cases, prostacyclin therapy is generally contraindicated due to the risk of worsening pulmonary edema. In this report, we described a patient with PCH and subsequent echocardiographic evidence of pulmonary hypertension, who experienced a worsening of respiratory symptoms that was precipitated by lower airway infection.

關鍵字

polymyositis, lung infiltrate ； pulmonary hypertension

並列摘要

肺微血管瘤病變（Pulmonary capillary hemangiomatosis）為一種罕見血管瘤病變，主要由肺部間質組織和血管被異常的微血管增生佔據而導致。臨床表現多樣，多以喘和肺高壓表現之。肺部影像上可見浸潤。此病需倚賴病理組織切片診斷。且不宜使用前列腺素類藥物治療其肺高壓。本報告描述一位中年女性，一開始因下呼吸道感染求診，臨床表現非典型，經鑑別診斷，最終確診有肺微血管瘤病變以及心臟超音波下的肺動脈高壓變化。

並列關鍵字

多發性肌炎 ； 肺間質浸潤 ； 肺動脈高壓