Please wait a minute...
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
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
Download: PDF(549 KB)   HTML
Export: BibTeX | EndNote | Reference Manager | ProCite | RefWorks

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.,   
Issue Date: 05 September 2013
URL:     OR
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
Some infections including coccidioidomycosis, pneumocystis jiroveci
Tab.1  Causes of diffuse cystic lung disease
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.
1 Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology 2008; 246(3): 697–722
doi: 10.1148/radiol.2462070712 pmid:18195376
2 Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc 2003; 78(6): 744–752
doi: 10.4065/78.6.744 pmid:12934786
3 Koyama M, Johkoh T, Honda O, Tsubamoto M, Kozuka T, Tomiyama N, Hamada S, Nakamura H, Akira M, Ichikado K, Fujimoto K, Rikimaru T, Tateishi U, Müller NL. Chronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients. AJR Am J Roentgenol 2003; 180(3): 827–835
doi: 10.2214/ajr.180.3.1800827 pmid:12591705
4 Vourtsi A, Gouliamos A, Moulopoulos L, Papacharalampous X, Chatjiioannou A, Kehagias D, Lamki N. CT appearance of solitary and multiple cystic and cavitary lung lesions. Eur Radiol 2001; 11(4): 612–622
doi: 10.1007/s003300000583 pmid:11354756
5 Woodring JH, Fried AM, Chuang VP. Solitary cavities of the lung: diagnostic implications of cavity wall thickness. AJR Am J Roentgenol 1980; 135(6): 1269–1271
doi: 10.2214/ajr.135.6.1269 pmid:6779538
6 Meraj R, Wikenheiser-Brokamp KA, Young LR, McCormack FX. Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment. Semin Respir Crit Care Med 2012; 33(5): 486–497
doi: 10.1055/s-0032-1325159 pmid:23001803
7 Zhang X, Travis WD. Pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med 2010; 134(12): 1823–1828
8 Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, Reynaud-Gaubert M, Boehler A, Brauner M, Popper H, Bonetti F, Kingswood C; Review Panel of the ERS LAM Task Force.European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 2010; 35(1): 14–26
doi: 10.1183/09031936.00076209 pmid:20044458
9 Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, Finlay GA, Olson EJ, Ruoss SJ, Maurer JR, Raffin TA, Peavy HH, McCarthy K, Taveira-Dasilva A, McCormack FX, Avila NA, Decastro RM, Jacobs SS, Stylianou M, Fanburg BL; NHLBI LAM Registry Group. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. [See comment.] Am J Respir Crit Care Med 2006; 173(1): 105–111
doi: 10.1164/rccm.200409-1298OC pmid:16210669
10 McCormack FX, Travis WD, Colby TV, Henske EP, Moss J. Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm. Am J Respir Crit Care Med 2012; 186(12): 1210–1212
doi: 10.1164/rccm.201205-0848OE pmid:23250499
11 Kumasaka T, Seyama K, Mitani K, Sato T, Souma S, Kondo T, Hayashi S, Minami M, Uekusa T, Fukuchi Y, Suda K. Lymphangiogenesis in lymphangioleiomyomatosis: its implication in the progression of lymphangioleiomyomatosis. Am J Surg Pathol 2004; 28(8): 1007–1016
doi: 10.1097/01.pas.0000126859.70814.6d pmid:15252306
12 Kumasaka T, Seyama K, Mitani K, Souma S, Kashiwagi S, Hebisawa A, Sato T, Kubo H, Gomi K, Shibuya K, Fukuchi Y, Suda K. Lymphangiogenesis-mediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis. Am J Surg Pathol 2005; 29(10): 1356–1366
doi: 10.1097/01.pas.0000172192.25295.45 pmid:16160479
13 Seyama K, Kumasaka T, Souma S, Sato T, Kurihara M, Mitani K, Tominaga S, Fukuchi Y. Vascular endothelial growth factor-D is increased in serum of patients with lymphangioleiomyomatosis. Lymphat Res Biol 2006; 4(3): 143–152
doi: 10.1089/lrb.2006.4.143 pmid:17034294
14 Young LR, Vandyke R, Gulleman PM, Inoue Y, Brown KK, Schmidt LS, Linehan WM, Hajjar F, Kinder BW, Trapnell BC, Bissler JJ, Franz DN, McCormack FX. Serum vascular endothelial growth factor-D prospectively distinguishes lymphangioleiomyomatosis from other diseases. Chest 2010; 138(3): 674–681
doi: 10.1378/chest.10-0573 pmid:20382711
15 Xu KF, Zhang P, Tian XT, Ma A, Li X, Zhou J, Zeng N, Gui YS, Guo Z, Feng R, Zhang W, Sun W, Cai B. The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis (LAM). Respir Med 2013; 107(2): 263–268
doi: 10.1016/j.rmed.2012.10.006 pmid:23127572
16 Seaman DM, Meyer CA, Gilman MD, McCormack FX. Diffuse cystic lung disease at high-resolution CT. AJR Am J Roentgenol 2011; 196(6): 1305–1311
doi: 10.2214/AJR.10.4420 pmid:21606293
17 Bonelli FS, Hartman TE, Swensen SJ, Sherrick A. Accuracy of high-resolution CT in diagnosing lung diseases. AJR Am J Roentgenol 1998; 170(6): 1507–1512
doi: 9609163" target="_blank">10.2214/ajr.170.6. pmid:9609163 pmid:9609163
18 Meraj R, Wikenheiser-Brokamp KA, Young LR, Byrnes S, McCormack FX. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis. Front Med 2012; 6(4): 395–405
doi: 10.1007/s11684-012-0231-5 pmid:23224417
19 Costello LC, Hartman TE, Ryu JH. High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex. Mayo Clin Proc 2000; 75(6): 591–594
doi: 10.4065/75.6.591 pmid:10852420
20 Franz DN, Brody A, Meyer C, Leonard J, Chuck G, Dabora S, Sethuraman G, Colby TV, Kwiatkowski DJ, McCormack FX. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001; 164(4): 661–668
doi: 10.1164/ajrccm.164.4.2011025 pmid:11520734
21 Moss J, Avila NA, Barnes PM, Litzenberger RA, Bechtle J, Brooks PG, Hedin CJ, Hunsberger S, Kristof AS. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med 2001; 164(4): 669–671
doi: 10.1164/ajrccm.164.4.2101154 pmid:11520735
22 McCormack FX, Inoue Y, Moss J, Singer LG, Strange C, Nakata K, Barker AF, Chapman JT, Brantly ML, Stocks JM, Brown KK, Lynch JP 3rd, Goldberg HJ, Young LR, Kinder BW, Downey GP, Sullivan EJ, Colby TV, McKay RT, Cohen MM, Korbee L, Taveira-DaSilva AM, Lee HS, Krischer JP, Trapnell BC; National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011; 364(17): 1595–1606
doi: 10.1056/NEJMoa1100391 pmid:21410393
23 Maurer JR, Ryu J, Beck G, Moss J, Lee JC, Finlay G, Brown K, Chapman J, McMahan J, Olson E, Ruoss S, Sherer S; National Heart, Lung, and Blood Institute LAM Registry Study Group. Lung transplantation in the management of patients with lymphangioleiomyomatosis: baseline data from the NHLBI LAM Registry. J Heart Lung Transplant 2007; 26(12): 1293–1299
doi: 10.1016/j.healun.2007.09.013 pmid:18096481
24 Benden C, Rea F, Behr J, Corris PA, Reynaud-Gaubert M, Stern M, Speich R, Boehler A. Lung transplantation for lymphangioleiomyomatosis: the European experience. J Heart Lung Transplant 2009; 28(1): 1–7
doi: 10.1016/j.healun.2008.09.014 pmid:19134523
25 Reynaud-Gaubert M, Mornex JF, Mal H, Treilhaud M, Dromer C, Quétant S, Leroy-Ladurie F, Guillemain R, Philit F, Dauriat G, Grenet D, Stern M. Lung transplantation for lymphangioleiomyomatosis: the French experience. Transplantation 2008; 86(4): 515–520
doi: 10.1097/TP.0b013e31817c15df pmid:18724219
26 Kpodonu J, Massad MG, Chaer RA, Caines A, Evans A, Snow NJ, Geha AS. The US experience with lung transplantation for pulmonary lymphangioleiomyomatosis. J Heart Lung Transplant 2005; 24(9): 1247–1253
doi: 10.1016/j.healun.2004.09.013 pmid:16143241
27 Vassallo R, Ryu JH. Smoking-related interstitial lung diseases. Clin Chest Med 2012; 33(1): 165–178
doi: 10.1016/j.ccm.2011.11.004 pmid:22365253
28 Allen TC. Pulmonary Langerhans cell histiocytosis and other pulmonary histiocytic diseases: a review. Arch Pathol Lab Med 2008; 132(7): 1171–1181
29 Vassallo R, Ryu JH, Colby TV, Hartman T, Limper AH. Pulmonary Langerhans’ cell histiocytosis. [See comment.] N Engl J Med 2000; 342(26): 1969–1978
doi: 10.1056/NEJM200006293422607 pmid:10877650
30 Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J 2006; 27(6): 1272–1285
doi: 10.1183/09031936.06.00024004 pmid:16772390
31 Vassallo R, Walters PR, Lamont J, Kottom TJ, Yi ES, Limper AH. Cigarette smoke promotes dendritic cell accumulation in COPD; a Lung Tissue Research Consortium study. Respir Res 2010; 11(1): 45
doi: 10.1186/1465-9921-11-45 pmid:20420706
32 Kambouchner M, Basset F, Marchal J, Uhl JF, Hance AJ, Soler P. Three-dimensional characterization of pathologic lesions in pulmonary langerhans cell histiocytosis. Am J Respir Crit Care Med 2002; 166(11): 1483–1490
doi: 10.1164/rccm.2201050 pmid:12450935
33 Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH. Pneumothorax in pulmonary Langerhans cell histiocytosis. Chest 2004; 125(3): 1028–1032
doi: 10.1378/chest.125.3.1028 pmid:15006964
34 Friedman PJ, Liebow AA, Sokoloff J. Eosinophilic granuloma of lung. Clinical aspects of primary histiocytosis in the adult. Medicine (Baltimore) 1981; 60(6): 385–396
doi: 10.1097/00005792-198111000-00001 pmid:6975871
35 Basset F, Corrin B, Spencer H, Lacronique J, Roth C, Soler P, Battesti JP, Georges R, Chrétien J. Pulmonary histiocytosis X. Am Rev Respir Dis 1978; 118(5): 811–820
36 Travis WD, Borok Z, Roum JH, Zhang J, Feuerstein I, Ferrans VJ, Crystal RG. Pulmonary Langerhans cell granulomatosis (histiocytosis X). A clinicopathologic study of 48 cases. Am J Surg Pathol 1993; 17(10): 971–986
doi: 10.1097/00000478-199310000-00002 pmid:8372949
37 Von Essen S, West W, Sitorius M, Rennard SI. Complete resolution of roentgenographic changes in a patient with pulmonary histiocytosis X. Chest 1990; 98(3): 765–767
doi: 10.1378/chest.98.3.765 pmid:2394160
38 Mogulkoc N, Veral A, Bishop PW, Bayindir U, Pickering CA, Egan JJ. Pulmonary Langerhans’ cell histiocytosis: radiologic resolution following smoking cessation. Chest 1999; 115(5): 1452–1455
doi: 10.1378/chest.115.5.1452 pmid:10334170
39 Aerni MR, Aubry MC, Myers JL, Vassallo R. Complete remission of nodular pulmonary Langerhans cell histiocytosis lesions induced by 2-chlorodeoxyadenosine in a non-smoker. Respir Med 2008; 102(2): 316–319
doi: 10.1016/j.rmed.2007.09.002 pmid:17935961
40 Lazor R, Etienne-Mastroianni B, Khouatra C, Tazi A, Cottin V, Cordier JF. Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy. Thorax 2009; 64(3): 274–275
doi: 10.1136/thx.2008.108944 pmid:19252036
41 Lorillon G, Bergeron A, Detourmignies L, Jouneau S, Wallaert B, Frija J, Tazi A. Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. Am J Respir Crit Care Med 2012; 186(9): 930–932
doi: 10.1164/ajrccm.186.9.930 pmid:23118088
42 Dauriat G, Mal H, Thabut G, Mornex JF, Bertocchi M, Tronc F, Leroy-Ladurie F, Dartevelle P, Reynaud-Gaubert M, Thomas P, Pison C, Blin D, Stern M, Bonnette P, Dromer C, Velly JF, Brugière O, Lesèche G, Fournier M. Lung transplantation for pulmonary langerhans’ cell histiocytosis: a multicenter analysis. Transplantation 2006; 81(5): 746–750
doi: 10.1097/ pmid:16534477
43 Liebow AA, Carrington CB. Diffuse pulmonary lymphoreticular infiltrations associated with dysproteinemia. Med Clin North Am 1973; 57(3): 809–843
44 Johkoh T, Müller NL, Pickford HA, Hartman TE, Ichikado K, Akira M, Honda O, Nakamura H. Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 1999; 212(2): 567–572
45 Swigris JJ, Berry GJ, Raffin TA, Kuschner WG. Lymphoid interstitial pneumonia: a narrative review. Chest 2002; 122(6): 2150–2164
doi: 10.1378/chest.122.6.2150 pmid:12475860
46 Cha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J 2006; 28(2): 364–369
doi: 10.1183/09031936.06.00076705 pmid:16571614
47 Tian X, Yi ES, Ryu JH. Lymphocytic interstitial pneumonia and other benign lymphoid disorders. Semin Respir Crit Care Med 2012; 33(5): 450–461
doi: 10.1055/s-0032-1325156 pmid:23001800
48 American Thoracic Society, European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. [Erratum appears in Am J Respir Crit Care Med 2002;166(3):426]. Am J Respir Crit Care Med 2002; 165(2): 277–304
doi: 10.1164/ajrccm.165.2.ats01 pmid:11790668
49 Ichikawa Y, Kinoshita M, Koga T, Oizumi K, Fujimoto K, Hayabuchi N. Lung cyst formation in lymphocytic interstitial pneumonia: CT features. J Comput Assist Tomogr 1994; 18(5): 745–748
doi: 10.1097/00004728-199409000-00012 pmid:8089323
50 Honda O, Johkoh T, Ichikado K, Tomiyama N, Maeda M, Mihara N, Higashi M, Hamada S, Naito H, Yamamoto S, Nakamura H. Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT. AJR Am J Roentgenol 1999; 173(1): 71–74
doi: 10397102" target="_blank">10.2214/ajr.173.1. pmid:10397102 pmid:10397102
51 Silva CIS, Flint JD, Levy RD, Müller NL. Diffuse lung cysts in lymphoid interstitial pneumonia: high-resolution CT and pathologic findings. J Thorac Imaging 2006; 21(3): 241–244
doi: 10.1097/01.rti.0000213554.61752.73 pmid:16915074
52 Parker JS, Shellito J, Pei LA, Mason CM. Lymphocytic interstitial pneumonitis presenting as recurrent pneumothoraces. Chest 1991; 100(6): 1733–1735
doi: 10.1378/chest.100.6.1733 pmid:1959426
53 Adley BP, Smith ND, Nayar R, Yang XJ. Birt-Hogg-Dubé syndrome: clinicopathologic findings and genetic alterations. Arch Pathol Lab Med 2006; 130(12): 1865–1870
54 Menko FH, van Steensel MAM, Giraud S, Friis-Hansen L, Richard S, Ungari S, Nordenskj?ld M, Hansen TV, Solly J, Maher ER; European BHD Consortium.Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol 2009; 10(12): 1199–1206
doi: 10.1016/S1470-2045(09)70188-3 pmid:19959076
55 Ayo DS, Aughenbaugh GL, Yi ES, Hand JL, Ryu JH. Cystic lung disease in Birt-Hogg-Dubé syndrome. Chest 2007; 132(2): 679–684
doi: 10.1378/chest.07-0042 pmid:17505035
56 Butnor KJ, Guinee DG Jr. Pleuropulmonary pathology of Birt-Hogg-Dubé syndrome. Am J Surg Pathol 2006; 30(3): 395–399
57 Toro JR, Pautler SE, Stewart L, Glenn GM, Weinreich M, Toure O, Wei MH, Schmidt LS, Davis L, Zbar B, Choyke P, Steinberg SM, Nguyen DM, Linehan WM. Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndrome. Am J Respir Crit Care Med 2007; 175(10): 1044–1053
doi: 10.1164/rccm.200610-1483OC pmid:17322109
58 Agarwal PP, Gross BH, Holloway BJ, Seely J, Stark P, Kazerooni EA. Thoracic CT findings in Birt-Hogg-Dubé syndrome. AJR Am J Roentgenol 2011; 196(2): 349–352
doi: 10.2214/AJR.10.4757 pmid:21257886
59 Houweling AC, Gijezen LM, Jonker MA, van Doorn MBA, Oldenburg RA, van Spaendonck-Zwarts KY, Leter EM, van Os TA, van Grieken NC, Jaspars EH, de Jong MM, Bongers EM, Johannesma PC, Postmus PE, van Moorselaar RJ, van Waesberghe JH, Starink TM, van Steensel MA, Gille JJ, Menko FH. Renal cancer and pneumothorax risk in Birt-Hogg-Dubé syndrome; an analysis of 115 FLCN mutation carriers from 35 BHD families. Br J Cancer 2011; 105(12): 1912–1919
doi: 10.1038/bjc.2011.463 pmid:22146830
60 Medvetz DA, Khabibullin D, Hariharan V, Ongusaha PP, Goncharova EA, Schlechter T, Darling TN, Hofmann I, Krymskaya VP, Liao JK, Huang H, Henske EP. Folliculin, the product of the Birt-Hogg-Dubé tumor suppressor gene, interacts with the adherens junction protein p0071 to regulate cell-cell adhesion. PLoS ONE 2012; 7(11): e47842
doi: 10.1371/journal.pone.0047842 pmid:23139756
61 Hasumi Y, Baba M, Ajima R, Hasumi H, Valera VA, Klein ME, Haines DC, Merino MJ, Hong SB, Yamaguchi TP, Schmidt LS, Linehan WM. Homozygous loss of BHD causes early embryonic lethality and kidney tumor development with activation of mTORC1 and mTORC2. Proc Natl Acad Sci USA 2009; 106(44): 18722–18727
doi: 10.1073/pnas.0908853106 pmid:19850877
62 Hartman TR, Nicolas E, Klein-Szanto A, Al-Saleem T, Cash TP, Simon MC, Henske EP. The role of the Birt-Hogg-Dubé protein in mTOR activation and renal tumorigenesis. Oncogene 2009; 28(13): 1594–1604
doi: 10.1038/onc.2009.14 pmid:19234517
63 Baba M, Hong SB, Sharma N, Warren MB, Nickerson ML, Iwamatsu A, Esposito D, Gillette WK, Hopkins RF 3rd, Hartley JL, Furihata M, Oishi S, Zhen W, Burke TR Jr, Linehan WM, Schmidt LS, Zbar B. Folliculin encoded by the BHD gene interacts with a binding protein, FNIP1, and AMPK, and is involved in AMPK and mTOR signaling. Proc Natl Acad Sci USA 2006; 103(42): 15552–15557
doi: 10.1073/pnas.0603781103 pmid:17028174
64 Furuya M, Tanaka R, Koga S, Yatabe Y, Gotoda H, Takagi S, Hsu YH, Fujii T, Okada A, Kuroda N, Moritani S, Mizuno H, Nagashima Y, Nagahama K, Hiroshima K, Yoshino I, Nomura F, Aoki I, Nakatani Y. Pulmonary cysts of Birt-Hogg-Dubé syndrome: a clinicopathologic and immunohistochemical study of 9 families. Am J Surg Pathol 2012; 36(4): 589–600
doi: 10.1097/PAS.0b013e3182475240 pmid:22441547
65 Tobino K, Hirai T, Johkoh T, Kurihara M, Fujimoto K, Tomiyama N, Mishima M, Takahashi K, Seyama K. Differentiation between Birt-Hogg-Dubé syndrome and lymphangioleiomyomatosis: quantitative analysis of pulmonary cysts on computed tomography of the chest in 66 females. Eur J Radiol 2012; 81(6): 1340–1346
doi: 10.1016/j.ejrad.2011.03.039 pmid:21550193
66 Tobino K, Gunji Y, Kurihara M, Kunogi M, Koike K, Tomiyama N, Johkoh T, Kodama Y, Iwakami S, Kikkawa M, Takahashi K, Seyama K. Characteristics of pulmonary cysts in Birt-Hogg-Dubé syndrome: thin-section CT findings of the chest in 12 patients. Eur J Radiol 2011; 77(3): 403–409
doi: 10.1016/j.ejrad.2009.09.004 pmid:19782489
67 Tomassetti S, Carloni A, Chilosi M, Maffè A, Ungari S, Sverzellati N, Gurioli C, Casoni G, Romagnoli M, Gurioli C, Ravaglia C, Poletti V. Pulmonary features of Birt-Hogg-Dubé syndrome: cystic lesions and pulmonary histiocytoma. Respir Med 2011; 105(5): 768–774
doi: 10.1016/j.rmed.2011.01.002 pmid:21356586
68 Gertz MA, Dispenzieri A. Immunoglobulin light-chain amyloidosis: growing recognition, new approaches to therapy, active clinical trials. Oncology (Williston Park) 2012; 26(2): 152–161
69 Gillmore JD, Hawkins PN. Amyloidosis and the respiratory tract. Thorax 1999; 54(5): 444–451
doi: 10.1136/thx.54.5.444 pmid:10212113
70 Lachmann HJ, Hawkins PN. Amyloidosis and the lung. Chron Respir Dis 2006; 3(4): 203–214
doi: 10.1177/1479972306070066 pmid:17190124
71 Lantuejoul S, Moulai N, Quetant S, Brichon PY, Brambilla C, Brambilla E, Ferretti GR. Unusual cystic presentation of pulmonary nodular amyloidosis associated with MALT-type lymphoma. Eur Respir J 2007; 30(3): 589–592
doi: 10.1183/09031936.00136605 pmid:17766635
72 Sakai M, Yamaoka M, Kawaguchi M, Hizawa N, Sato Y. Multiple cystic pulmonary amyloidosis. Ann Thorac Surg 2011; 92(5): e109
doi: 10.1016/j.athoracsur.2011.07.063 pmid:22051316
73 Kobayashi H, Matsuoka R, Kitamura S, Tsunoda N, Saito K. Sj?gren’s syndrome with multiple bullae and pulmonary nodular amyloidosis. Chest 1988; 94(2): 438–440
doi: 10.1378/chest.94.2.438 pmid:3396429
74 Jeong YJ, Lee KS, Chung MP, Han J, Chung MJ, Kim KI, Seo JB, Franquet T. Amyloidosis and lymphoproliferative disease in Sj?gren syndrome: thin-section computed tomography findings and histopathologic comparisons. J Comput Assist Tomogr 2004; 28(6): 776–781
doi: 10.1097/00004728-200411000-00008 pmid:15538150
75 Ohdama S, Akagawa S, Matsubara O, Yoshizawa Y. Primary diffuse alveolar septal amyloidosis with multiple cysts and calcification. Eur Respir J 1996; 9(7): 1569–1571
doi: 10.1183/09031936.96.09071569 pmid:8836676
76 Yanagawa N, Ogata SY, Motoyama T. Pulmonary localized AA type amyloidosis with cyst-like structures and marginal zone B-cell lymphoma of the MALT type coexisting independently in the left upper lung. Intern Med 2008; 47(17): 1529–1533
doi: 10.2169/internalmedicine.47.1231 pmid:18758129
77 Colombat M, Stern M, Groussard O, Droz D, Brauner M, Valeyre D, Mal H, Taillé C, Monnet I, Fournier M, Herson S, Danel C. Pulmonary cystic disorder related to light chain deposition disease. Am J Respir Crit Care Med 2006; 173(7): 777–780
doi: 10.1164/rccm.200510-1620CR pmid:16399989
78 Bhargava P, Rushin JM, Rusnock EJ, Hefter LG, Franks TJ, Sabnis SG, Travis WD. Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol 2007; 31(2): 267–276
doi: 10.1097/01.pas.0000213358.18380.d2 pmid:17255772
79 Colombat M, Caudroy S, Lagonotte E, Mal H, Danel C, Stern M, Fournier M, Birembaut P. Pathomechanisms of cyst formation in pulmonary light chain deposition disease. Eur Respir J 2008; 32(5): 1399–1403
doi: 10.1183/09031936.00132007 pmid:18978139
80 Collins J. CT signs and patterns of lung disease. Radiol Clin North Am 2001; 39(6): 1115–1135
doi: 10.1016/S0033-8389(05)70334-1 pmid:11699664
81 Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest 2005; 127(1): 178–184
doi: 10.1378/chest.127.1.178 pmid:15653981
82 Ryu JH, Colby TV, Hartman TE, Vassallo R. Smoking-related interstitial lung diseases: a concise review. Eur Respir J 2001; 17(1): 122–132
doi: 10.1183/09031936.01.17101220 pmid:11307741
83 Akira M, Yamamoto S, Hara H, Sakatani M, Ueda E. Serial computed tomographic evaluation in desquamative interstitial pneumonia. Thorax 1997; 52(4): 333–337
doi: 10.1136/thx.52.4.333 pmid:9196515
84 Franquet T, Hansell DM, Senbanjo T, Remy-Jardin M, Müller NL. Lung cysts in subacute hypersensitivity pneumonitis. J Comput Assist Tomogr 2003; 27(4): 475–478
doi: 10.1097/00004728-200307000-00003 pmid:12886127
85 Colby TV. Bronchiolitis. Pathologic considerations. Am J Clin Pathol 1998; 109(1): 101–109
86 Ryu JH, Myers JL, Swensen SJ. Bronchiolar disorders. Am J Respir Crit Care Med 2003; 168(11): 1277–1292
doi: 10.1164/rccm.200301-053SO pmid:14644923
87 Devakonda A, Raoof S, Sung A, Travis WD, Naidich D. Bronchiolar disorders: a clinical-radiological diagnostic algorithm. Chest 2010; 137(4): 938–951
doi: 10.1378/chest.09-0800 pmid:20371529
88 Lynch DA. Imaging of small airways disease and chronic obstructive pulmonary disease. Clin Chest Med 2008; 29(1): 165–179, vii
doi: 10.1016/j.ccm.2007.11.008 pmid:18267190
89 Hansell DM. HRCT of obliterative bronchiolitis and other small airways diseases. Semin Roentgenol 2001; 36(1): 51–65
doi: 10.1053/sroe.2001.20463 pmid:11204759
90 Howling SJ, Hansell DM, Wells AU, Nicholson AG, Flint JD, Müller NL. Follicular bronchiolitis: thin-section CT and histologic findings. Radiology 1999; 212(3): 637–642
91 Kawano-Dourado L, Baldi BG, Dias OM, Bernardi FDC, Carvalho CRR, Dolhnikoff M, Kairalla RA. Scattered lung cysts as the main radiographic finding of constrictive bronchiolitis. Am J Respir Crit Care Med 2012; 186(3): 294–295
doi: 10.1164/ajrccm.186.3.294 pmid:22855547
92 Rowan C, Hansell DM, Renzoni E, Maher TM, Wells AU, Polkey MI, Rehal PK, Ibrahim WH, Kwong GN, Colby TV, Pistolesi M, Bigazzi F, Comin CE, Nicholson AG. Diffuse cystic lung disease of unexplained cause with coexistent small airway disease: a possible causal relationship? Am J Surg Pathol 2012; 36(2): 228–234
doi: 10.1097/PAS.0b013e318237c599 pmid:22082605
93 Karpman C, Aughenbaugh GL, Ryu JH. Pneumothorax and bullae in Marfan syndrome. Respiration 2011; 82(3): 219–224
doi: 10.1159/000322958 pmid:21252480
94 Ryu JH, Parambil JG, McGrann PS, Aughenbaugh GL. Lack of evidence for an association between neurofibromatosis and pulmonary fibrosis. Chest 2005; 128(4): 2381–2386
doi: 10.1378/chest.128.4.2381 pmid:16236898
95 Zamora AC, Collard HR, Wolters PJ, Webb WR, King TE. Neurofibromatosis-associated lung disease: a case series and literature review. Eur Respir J 2007; 29(1): 210–214
doi: 10.1183/09031936.06.00044006 pmid:16870664
96 Murray RA, Poulton TB, Saltarelli MG, Dweik RA, Litwin DK, Kirby TJ, Meziane MA, O’Donovan PB. Rare pulmonary manifestation of Ehlers-Danlos syndrome. J Thorac Imaging 1995; 10(2): 138–141
doi: 10.1097/00005382-199521000-00012 pmid:7769630
97 Dowton SB, Pincott S, Demmer L. Respiratory complications of Ehlers-Danlos syndrome type IV. Clin Genet 1996; 50(6): 510–514
doi: 10.1111/j.1399-0004.1996.tb02724.x pmid:9147885
98 Irion KL, Hocchegger B, Marchiori E, Holemans JA, Smith RA, Raja RC, Singanayagam S. Proteus syndrome: high-resolution CT and CT pulmonary densitovolumetry findings. J Thorac Imaging 2009; 24(1): 45–48
doi: 10.1097/RTI.0b013e31818b20cd pmid:19242304
99 Lim GY, Kim OH, Kim HW, Lee KS, Kang KH, Song HR, Cho TJ. Pulmonary manifestations in Proteus syndrome: pulmonary varicosities and bullous lung disease. Am J Med Genet A 2011; 155A(4): 865–869
doi: 10.1002/ajmg.a.33926 pmid:21412980
100 Hage CA, Knox KS, Wheat LJ. Endemic mycoses: overlooked causes of community acquired pneumonia. Respir Med 2012; 106(6): 769–776
doi: 10.1016/j.rmed.2012.02.004 pmid:22386326
101 Thompson GR 3rd. Pulmonary coccidioidomycosis. Semin Respir Crit Care Med 2011; 32(6): 754–763
doi: 10.1055/s-0031-1295723 pmid:22167403
102 Catherinot E, Lanternier F, Bougnoux ME, Lecuit M, Couderc LJ, Lortholary O. Pneumocystis jirovecii Pneumonia. Infect Dis Clin North Am 2010; 24(1): 107–138
doi: 10.1016/j.idc.2009.10.010 pmid:20171548
103 Reid AB, Chen SCA, Worth LJ. Pneumocystis jirovecii pneumonia in non-HIV-infected patients: new risks and diagnostic tools. Curr Opin Infect Dis 2011; 24(6): 534–544
doi: 10.1097/QCO.0b013e32834cac17 pmid:21986616
104 Kanne JP, Yandow DR, Meyer CA. Pneumocystis jiroveci pneumonia: high-resolution CT findings in patients with and without HIV infection. AJR Am J Roentgenol 2012; 198(6): W555–561
doi: 10.2214/AJR.11.7329 pmid:22623570
105 Kluka EM, Bauer PR, Aubry MC, Ryu JH. Enlarging lung nodules and cysts in a 53-year-old woman with primary Sj?gren syndrome. Chest 2013; 143(1): 258–261
doi: 10.1378/chest.12-0547 pmid:23276851
106 Traweek T, Rotter AJ, Swartz W, Azumi N. Cystic pulmonary metastatic sarcoma. Cancer 1990; 65(8): 1805–1811
doi: 10.1002/1097-0142(19900415)65:8&lt;1805::AID-CNCR2820650823&gt;3.0.CO;2-V pmid:2156605
107 Marti-Bonmati L, Catala FJ, Ruiz Perales F. Computed tomography differentiation between cystic bronchiectasis and bullae. J Thorac Imaging 1991; 7(1): 83–85
doi: 10.1097/00005382-199112000-00011 pmid:1779449
108 McGuinness G, Naidich DP. CT of airways disease and bronchiectasis. Radiol Clin North Am 2002; 40(1): 1–19
doi: 10.1016/S0033-8389(03)00105-2 pmid:11813813
109 Matsui K, Takeda K, Yu ZX, Travis WD, Moss J, Ferrans VJ. Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med 2000; 124(2): 267–275
[1] Vinay K. Kapoor. Bile duct injury repair — earlier is not better[J]. Front. Med., 2015, 9(4): 508-511.
[2] Jeffrey H. Mandel,Christine Wendt,Charles Lo,Guangbiao Zhou,Marshall Hertz,Gurumurthy Ramachandran. Ambient air pollution and lung disease in China: health effects, study design approaches and future research[J]. Front. Med., 2015, 9(3): 392-400.
[3] Xianghu Meng, Jihong Liu, Xiao Yu, Shaogang Wang, Cong Liu, Zhangqun Ye. A giant Müllerian duct cyst in the perineum: a case report[J]. Front Med, 2012, 6(4): 440-443.
[4] Riffat Meraj, Kathryn A. Wikenheiser-Brokamp, Lisa R. Young, Sue Byrnes, Francis X. McCormack. Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis[J]. Front Med, 2012, 6(4): 395-405.
[5] Hongyi Cui. Single incision laparoscopic cholecystectomy using the one-incision three-trocar technique with all straight instruments: how I do it?[J]. Front Med, 2011, 5(3): 283-287.
[6] Hao WEN, Tuerganaili AJI, Ying-Mei SHAO, . Diagnosis and management against the complications of human cystic echinococcosis[J]. Front. Med., 2010, 4(4): 394-398.
[7] Zhi-Yong ZHANG, Xiao-Ping CHEN, Qi-Ping LU. Effect of salvia miltiorrhiza pretreatment on the CCK and VIP expression in hepatic ischemia-reperfusion-induced digestive tract congestion[J]. Front Med Chin, 2010, 4(3): 317-322.
[8] Zi-Jiang CHEN, Yuhua SHI. Polycystic ovary syndrome[J]. Front Med Chin, 2010, 4(3): 280-284.
[9] Yuanyuan LIN MD, Xiaoming CHENG MS, Yuhu SONG MD, Peiyuan LI MD, Ying CHANG MM, Jinjian YAO MD, Jusheng LIN MD, PhD, Li ZHOU PhD, Leiming XU PhD, . Construction and expression of hepatitis B virus vector encoding TC-tagged core protein[J]. Front. Med., 2009, 3(4): 396-402.
[10] YAN Jiangtao, SHAO Jiaomei, WANG Daowen, YUE Zhengliang, HUI Rutai. Predication of increased plasma homocysteine level on the prognosis of Chinese patients with first-onset ischemic stroke[J]. Front. Med., 2008, 2(4): 352-355.
[11] LIU Anzhong, LI Jun, HUA Huwei. Beneficial effects of preventive cholecystectomy in patients with hepatic cancer[J]. Front. Med., 2008, 2(2): 139-142.
[12] WANG Lin, LIU Yalan, XU Jianbo, TIAN Yuan, WU Heshui. Protective effect of N-acetylcysteine against lipopolysaccharide injury in hepatocytes of neonatal mice[J]. Front. Med., 2008, 2(2): 182-185.
[13] GAO Yan, CHEN Yan, SUN Zhipeng, GUO Chuanbin, YU Guangyan, ZHAN Yi. Diagnosis and treatment of thyroglossal duct carcinoma: Report of three cases with review of literatures[J]. Front. Med., 2008, 2(1): 58-62.
[14] ZHU Wenyu, TAN Liping, CHEN Xiangfeng, HUANG Qiang, LAN Qing. High risk factors for pulmonary fungous infection in intensive care units of neurosurgery[J]. Front. Med., 2007, 1(3): 299-303.
[15] LOU Wenhui, JIN Dayong, WANG Dansong, XU Xuefeng, KUANG Tiantao, QIN Xinyu. An analysis of clinico-pathologic features of intraductal papillary mucinous neoplasm of the pancreas[J]. Front. Med., 2007, 1(2): 173-176.
Full text