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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front Med    2013, Vol. 7 Issue (3) : 316-327     DOI: 10.1007/s11684-013-0269-z
REVIEW |
Diffuse cystic lung diseases
Jay H. Ryu1(), Xinlun Tian2, Misbah Baqir1, Kaifeng Xu2
1. Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA; 2. Division of Respiratory Medicine, Peking Union Medical College Hospital, Beijing 100730, China
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Abstract  

Diffuse cystic lung diseases are uncommon but can present a diagnostic challenge because increasing number of diseases have been associated with this presentation. Cyst in the lung is defined as a round parenchymal lucency with a well-defined thin wall (<2 mm thickness). Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes such as coccidioidomycosis, Pneumocystis jiroveci pneumonia, and hydatid disease. “Diffuse” distribution in the lung implies involvement of all lobes. Diffuse lung involvement with cystic lesions can be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans’ cell histiocytosis, lymphoid interstitial pneumonia, Birt-Hogg-Dubé syndrome, amyloidosis, light chain deposition disease, honeycomb lung associated with advanced fibrosis, and several other rare causes including metastatic disease. High-resolution computed tomography of the chest helps define morphologic features of the lung lesions as well as their distribution and associated features such as intrathoracic lymphadenopathy. Correlating the tempo of the disease process and clinical context with chest imaging findings serve as important clues to defining the underlying nature of the cystic lung disease and guide diagnostic evaluation as well as management.

Keywords cyst      lung disease      interstitial lung disease      lymphangioleiomyomatosis      Langerhans’ cell histiocytosis      Birt-Hogg-Dubé syndrome     
Corresponding Authors: Ryu Jay H.,Email:ryu.jay@mayo.edu   
Issue Date: 05 September 2013
URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-013-0269-z     OR     http://academic.hep.com.cn/fmd/EN/Y2013/V7/I3/316
Major causes
Lymphangioleiomyomatosis (LAM)
Pulmonary Langerhans’ cell histiocytosis (PLCH)
Lymphoid interstitial pneumonia (LIP) *
Birt-Hogg-Dubé syndrome (BHD)
Amyloidosis ?
Rarer causes
Light chain deposition disease (LCDD)
Follicular bronchiolitis
Metastatic disease, e.g., sarcoma
Several hereditary syndromes including Marfan syndrome, neurofibromatosis 1, Ehlers-Danlos syndrome, and Proteus syndrome
Disorders with cystic lesions as a component
Advanced fibrotic lung diseases, e.g., idiopathic pulmonary fibrosis, sarcoidosis
Desquamative interstitial pneumonia
Hypersensitivity pneumonitis
Emphysema
Some infections including coccidioidomycosis, pneumocystis jiroveci
Tab.1  Causes of diffuse cystic lung disease
LAMPLCHLIPBHDAmyloidosis
Age at diagnosisAdultsYounger adults, 3rd and 4th decadesBroad rangeAdultsAdults
GenderNearly all womenNo predominanceNo predominanceNo predominanceNo predominance
Relevant historyPneumothorax in majority; some have underlying TSCNearly all have smoking history; pneumothorax in 15%Some have underlying CTD, immunodeficiency, etc.Pneumothorax in 15%–25%; family history of BHD, renal tumor or pneumothoraxSome have systemic amyloidosis or underlying disease, e.g., CTD
Extrapulmonary manifestationsRenal angiomyolipoma, chylous ascites, stigmata of TSCBone lesions, diabetes insipidusSigns of underlying disease, e.g., CTD, HIV infection, etc.Benign skin tumors, renal neoplasmsSigns of underlying systemic amyloidosis, CTD, etc.
Laboratory testingElevated serum VEGF-D level, genetic testing (TSC)NADysproteinemia (polyclonal)Genetic testing (FLCN gene mutations)Dysproteinemia (monoclonal)
HRCT findingsMany round cysts, mostly 2 mm to 2 cm in size, with normal intervening lung parenchyma; diffuse distributionIrregular cysts, often with nodules and architectural distortion of intervening parenchyma; relative sparing of lung basesCysts of varying sizes and shapes, often with ground-glass opacities, nodules, septal thickening and lymphadenopathyCysts of varying sizes and shapes; more prominent in lower lobesScattered cysts of varying sizes, often with nodules
Tab.2  Main characteristics of five major diffuse cystic lung diseases
Fig.1  High-resolution CT scan of 35-year-old woman, nonsmoker, with lymphangioleiomyomatosis. Round cystic lesions are distributed symmetrically throughout both lung fields, characteristic of this disease.
Fig.2  (A) High-resolution CT scan of a 31-year-old woman, smoker, with pulmonary Langerhans’ cell histiocytosis. There are multiple cystic lesions, some of irregular shape, in both lungs. In addition, there are many nodules in both lungs as well. (B Coronal view demonstrating prominent involvement of upper and mid lung zones with relative sparing of the lung bases.
Fig.3  High-resolution CT scan of a 73-year-old man, nonsmoker, with idiopathic lymphoid interstitial pneumonia. Irregularly-shaped cystic lesions are noted bilaterally along with patchy infiltrates in the right lung.
Fig.4  High-resolution CT scan of a 60-year-old man, ex-smoker, with Birt-Hogg-Dubé syndrome. Cystic lesions of varying sizes are present in both lungs with asymmetric distribution with otherwise normal-appearing lung parenchyma. This patient presented with abdominal discomfort related to a renal mass that proved to be a renal cell carcinoma and was incidentally noted to have cystic lung disease.
Fig.5  High resolution CT scan of a 56-year-old woman, nonsmoker, with AL amyloidosis and lung involvement. Bilateral pulmonary cysts of widely varying sizes and asymmetric distribution are noted.
Fig.6  CT scan of a 57-year-old man, ex-smoker, with light chain deposition disease. Cystic lesions are present bilaterally with some coalescing into irregular shapes (peripheral right lung). There are also scattered small nodules in both lungs as well as clustered nodules in the right lung posteromedially. This patient presented with progressive renal insufficiency related to light chain deposition disease involving the kidneys.
Fig.7  High-resolution CT scan of a 62-year-old man, ex-smoker, with idiopathic pulmonary fibrosis. Honeycombing is present predominantly involving the peripheral lung zones and bases, characteristic of this lung disease.
Fig.8  CT scan of a 62-year-old man, ex-smoker, with metastatic colon cancer. Multiple nodules, some cavitated, and cysts are present bilaterally.
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