彌漫性肺部囊腫病變

Diffuse Cystic Lung Disease

10.6314/JIMT.202002_31(1).05

林莞欣(Wan-Hsin Lin)；王鶴健(Hao-Chien Wang)

瀰漫性肺部囊腫病變（Diffuse cystic lung disease） ； 淋巴管平滑肌增生症（Lymphangioleiomyomatosis） ； 蘭格罕細胞組織球增生症（Langerhans cell histiocytosis） ； Birt-Hogg-Dubé syndrome ； 淋巴細胞間質性肺炎（Lymphocytic interstitial pneumonia）

內科學誌

31卷1期（2020 / 02 / 01）

18 - 29

繁體中文

瀰漫性肺部囊腫為影響每個肺葉的多發性囊腫病變，是種罕見的病徵。會有此表現的疾病通常較為慢性，在不同性別、年齡的族群中好發的病因亦不同。由於肺部高解析度電腦斷層影像檢查（high-resolution computed tomography, HRCT）的普及，對於瀰漫性肺部囊腫病變的了解亦逐漸增加。除了能有效偵測到囊腫外，影像上囊腫的形狀、大小、分布的狀況，是否有其他肺部病變，都是能幫助鑑別診斷的重要線索。臨床上較常遇到的瀰漫性肺部囊腫疾病包含淋巴管平滑肌增生症（lymphangioleiomyomatosis）、肺部蘭格罕細胞組織球增生症（pulmonary Langerhans cell histiocytosis）、Birt-Hogg-Dubé syndrome及淋巴細胞間質性肺炎（lymphocytic interstitial pneumonia），在流行病學、臨床表現與影像學上各具特色。遵循一定的診斷流程，大部分的病人都能獲得正確的診斷，而不需要接受切片檢查。由於瀰漫性肺部囊腫病變非常罕見，未來仍需要進一步去研究各個疾病的機轉、治療及預後。

Diffuse cystic lung disease represents a diverse group of uncommon disorders, which are characterized by cystic change in every lobe of the lung. They're usually chronic onset, and each disease has its distinct epidemiology and manifestation. Knowledge of diffuse cystic lung disease has increased in the past decade with the widespread use of high-resolution computed tomography (HRCT) imaging. The size, shape and distribution of the cysts as well as other associated findings offer useful information on diagnosis. The main diseases that are discussed in this review are lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, Birt-Hogg-Dubé syndrome and lymphocytic interstitial pneumonia. By using the images of the HRCT and incorporating the patient's clinical history, physical examination and laboratory findings, it likely makes a confident and accurate diagnosis without the need for surgical lung biopsy. Due to the rarity of cystic lung diseases, more research needs to be done to have better understanding of their pathophysiology and prognosis.