The Indian Journal of Chest Diseases and Allied Sciences
Volume 65 | Issue 1 | Year 2023

Clinico-etiological Characteristics of Cystic Lung Disease: A Retrospective Study

Priyanka Singh1https://orcid.org/0000-0002-2468-8394, Amit Singh Vasan2https://orcid.org/0000-0002-6698-0449, Nitin Balram Ahuja3, Saikat Bhattacharjee4, Manoj Gopal Madakshira5, Arun Hegde6

1Department of Pulmonology, Army Hospital, Delhi, India

2Department of Respiratory Medicine, Command Hospital, Chandigarh, India

3Department of Hospital Administration, Command Hospital, Lucknow, Uttar Pradesh, India

4Department of Radiodiagnosis, Command Hospital, Lucknow, Uttar Pradesh, India

5Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

6Department of Rheumatology, Command Hospital, Lucknow, Uttar Pradesh, India

Corresponding Author: Priyanka Singh, Department of Pulmonology, Army Hospital, Delhi, India, Phone: +91 9923678680, e-mail: priyankafiglu@gmail.com

How to cite this article: Singh P, Vasan AS, Ahuja NB, et al. Clinico-etiological Characteristics of Cystic Lung Disease: A Retrospective Study. Indian J Chest Dis Allied Sci 2023;65(1):32–38.

Source of support: Nil

Conflict of interest: None

Received on: 02 May 2023; Accepted on: 31 May 2023; Published on: 29 June 2023


Introduction: Diffuse cystic lung diseases (CLDs) are a heterogeneous group of uncommon disorders with characteristic imaging appearance. Cystic lung disease is a significant cause of mortality and morbidity with a wide spectrum of radiological presentations and etiological differentials. Though the literature is widely available on radiological approaches to CLD, a knowledge gap exists on the etiological spectrum, especially in the Indian scenario, as it is an orphan group of lung disorders. The interest and experience among pulmonologists regarding CLD are growing with the widespread use of CT scans. Clinical, radiographic, and histological findings are often essential for proper diagnosis, and multidisciplinary approach is required for optimal management of such cases. In our study, through real-world cases, we have highlighted the clinical manifestations and diverse etiological spectrum of CLD. Since these disorders are rare, incurable, and have variable disease progression, the authors have tried to address the holistic approach of this relatively less-understood group of disorders.

Aims and objectives: The aim of the study was to identify clinical characteristics and etiological spectrum of patients manifesting with diffuse cystic lung disease radiologically.

Materials and methods: In this retrospective analysis, the hospital electronic database was screened with Boolean operations and keywords for cysts OR pneumothorax. Among a total of 4,479 patients admitted to the respiratory ward /ICU during the period of January 2020–September 2022 at a tertiary care center in northern India, 14 patients with radiological diagnosis of CLD matched the relevant search. All relevant data of these patients were retrieved from the records.

Results: Our patients presented predominantly with symptoms of cough and breathlessness. About 4 patients had pneumothorax as the first presentation. The mean age of presentation was 42.14 (standard deviation 12.6, age range 16–62 years). About 64% (n = 9/15) patients were females. The various etiologies identified were lymphangioleiomyomatosis (LAM), lymphocytic interstitial pneumonia, Birt–Hogg–Dube syndrome, hypersensitivity pneumonitis, Pneumocystis jirovecii pneumonia, and cystic metastasis, and in one patient, no definite cause was found despite detailed evaluation.

Conclusion: Cystic lung disorders are a less commonly diagnosed entity with rare etiologies. In our study, we found a female preponderance and LAM as the commonest CLD. Pneumothorax is a sentinel event and commonly the presenting complaint in CLD. Identification of the etiology can help in institution of definite therapy when available. In view of unpredictable disease progression and outcome, these diseases warrant follow-up and imaging surveillance.

Keywords: Cyst, Lymphangioleiomyomatosis, Pneumothorax, Spirometry, Symptoms.


BAL = Bronchoalveolar lavage; BHD = Birt–Hogg–Dube; CLD = Cystic lung diseases; CT = Computed tomography; DIP = Desquamative interstitial pneumonia; FLCN-S = Folliculin gene-associated syndrome; HP = Hypersensitivity pneumonitis; HRCT = High-resolution computed tomography; LAM = Lymphangioleiomyomatosis; LCDD = Light-chain deposition disease; LIP = Lymphocytic interstitial pneumonia; MMP = Matrix metalloproteinase; PLCH = Pulmonary Langerhans cell histiocytosis; PJP = Pneumocystis jirovecii pneumonia; TSC = Tuberous sclerosis complex; TPC = Total pleural covering; VEGF = Vascular endothelial growth factor; VATS = Video-assisted thoracic surgery.


A pulmonary cyst is defined as a usually thin walled (usually < 2 mm), round lucency in lung parenchyma, or a low-attenuating area that has a well-defined interface with normal parenchyma of the lung on chest-computed tomography. Multiple pulmonary cysts can be due to a heterogeneous group of orphan lung diseases clubbed under the umbrella term “cystic lung disease” (CLD). Cystic lung disease needs to be differentiated from cavity, bleb, bullae, emphysema, honeycombing, and pneumatocele, all of which may mimic a cyst. The differentiating features for these lucencies maybe cyst wall thickness, size of air-filled space, the location, and the clustering tendency of true cysts.1 Awareness about CLDs has recently risen as a consequence to widespread use of high-resolution computed tomography (HRCT) in clinical practice among pulmonologists. Solitary or sporadic cysts can be commonly seen in otherwise-normal individuals. However, multiple pulmonary cysts can be part of various progressive lung diseases. Cysts are rarely found in individuals less than 55 years of age. However, an increase in prevalence is seen with age.2,3 Etiological heterogeneity exists with CLD. Based on the pathophysiological mechanism involved, CLD can be classified broadly as genetic, congenital, inflammatory, infectious, smoking-related, lymphoproliferative, and neoplastic.4,5 In this article, we analyze 14 cases retrospectively from a tertiary care center and evaluate the clinical spectrum of their presentation based on etiology.


This was a retrospective analysis. The hospital electronic database was screened with Boolean operations and keywords for cysts OR pneumothorax. Among a total of 4,479 patients admitted to a respiratory unit /ICU during the period of January 2020–September 2022 at a tertiary care center in northern India, 14 patients with radiological diagnosis of CLD matched the relevant search. All relevant data of these patients were retrieved from the records.

All cases in this study had undergone chest radiograph and HRCT of the chest. One millimeter thin scans were performed on all patients using a 256-slice computed tomography (CT) scanner (Briallance ICT, 256 slice, Philips).

The CT scans were analyzed by a radiologist trained in lung CTs. All CTs with diffuse cysts were considered. An emphasis was made to exclude cyst mimickers like emphysema, bullae, honeycombing, etc.3,6

The demographic characteristics, symptom profile of patients, laboratory data, serological analysis like vascular endothelial growth factor (VEGF), bronchoscopy and broncho-alveolar results, reports of lung biopsy, and data on follow-up visits were collected from the hospital patient records.

C-reactive protein, antinuclear antibodies, and VEGF were done on case-to-case basis. Transbronchial lung biopsy/video-assisted thoracic surgery (VATS) biopsy was performed on a case-to-case basis based on patient’s consent and risk of pneumothorax.

A multidisciplinary approach was resorted to when histopathological evidence was not available, and the diagnosis was arrived based on clinical profile, radiological features, and other systemic illness histories


In our study, 14 patients with diffuse CLDs were included. Table 1 depicts the salient characteristics of cases included in the study group and other relevant history and diagnostic modalities. The mean age of presentation was 42.14 (standard deviation 12.6, age range 16–62 years). About 64% (n = 9/15) patients were females and n = 5 were males.

Table 1: Clinical and etiological profile of patients
Sl. no. Age/Sex Etiology Clinical presentation Comorbidities/History S Mode of diagnosis Follow-up Outcome
1 52/F LAM Dyspnea (Pneumothorax) Renal angiomyolipoma N Renal biopsy, VEGF 1 year Recurrent pneumothorax
2 38/M PJP Cough, dyspnea HIV N BAL – PJP cysts, IFA 2 years Recovered
3 36/M BHD Dyspnea (Pneumothorax) Nil Y VATS biopsy, FLCN gene 6 months Recurrent pneumothorax
4 41/F LAM Asymptomatic Nil N Clinical profile + VEGF 1.5 years Stable
5 29/F LIP Hemoptysis Sjogrens N ANA, anti-Ro/SSA, and anti-La 2 years Stable
6 16/F PJP Dyspnea HIV N BAL – Cysts, IFA 2 years On LTOT
7 42/F BHD Dyspnea (Pneumothorax) Family history N TBLB, FLCN gene 1 year Recurrent pneumothorax
8 29/M HP Dyspnea H/O parakeets exposure N BAL lymphocytosis (60)%, TBLB granuloma 1.5 years Stable
9 51/F LAM Asymptomatic CKD on dialysis N Clinical + VEGF 1 year Stable
10 47/F LAM Cough, dyspnea Nil N Clinical + VEGF 1 year Stable
11 45/F PJP Dyspnea APML N BAL – PJP cysts, IFA 6 months Recovered
12 58/M Cystic mets Cough, dyspnea Colorectal cancer Y Case of cancer (biopsy proven) 3 months Deceased
13 44/F LAM Dyspnea (Pneumothorax) Nil N Clinical + VEGF 1.5 years Declining lung function
14 62/M No definite etiology Cough, dyspnea CAD Y 1 year Stable
APML, acute promyelocytic leukemia; BAL, bronchoalveolar lavage; BHD, Birt–Hogg–Dube, CAD, coronary artery disease; F, female, FLCN, folliculin gene; HIV, human immunodeficiency virus, HP, hypersensitivity pneumonitis; IFA, immunofluorescence; LAM, lymphangioleiomyomatosis, LIP, lymphocytic interstitial pneumonia; LTOT, long-term oxygen therapy; M, male; N, no; PJP, Pneumocystis jiroveccii; S, smoking; TBLB, transbronchial lung biopsy; VATS, video-assisted thoracoscopic biopsy; VEGF, vascular endothelial growth factors; Y, yes

Majority of the patients were symptomatic with dyspnea, n = 11/14, i.e., 78%, and cough was present in 28% (n = 4/14). One patient had hemoptysis at presentation, and four had pneumothorax at first presentation. Two patients were asymptomatic and were detected incidentally while evaluated for other conditions. Only three patients among 14 were smokers.

Spirometry and diffusion study data were available for 10 patients. About 40% (n = 4/10) patients had obstruction on spirometry, and n = 4 had no functional impairment. Five patients had shown diffusion impairment. Functional evaluation is depicted in Figure 1.

Fig. 1: Functional impairment in patients of cystic lung disease. BHD, Birt–Hogg–Dube; HP, hypersensitivity pneumonitis; LAM, lymphangioleiomyomatosis; LIP, lymphocytic interstitial pneumonia; PJP, Pneumocystis jirovecii pneumonia

Radiologically, cysts were seen bilaterally in all patients except one who had unilateral cysts. In addition, one CT showed nodules and one CT showed associated ground glassing. None of the CT scans had shown mediastinal lymphadenopathy. Patients with pneumothorax underwent ICD insertion and pleurodesis with talc. Three patients had recurrent pneumothorax during follow-up. All patients with lymphangioleiomyomatosis (LAM) were started on sirolimus. All patients were counseled regarding symptomatology of pneumothorax and advised to seek immediate medical attention in such scenarios and regular follow-up.

Few representative images are depicted in Figures 2 and 6. Figure 2 shows CT image and bronchoalveolar lavage microscopy of a case of Pneumocystis jirovecii pneumonia (PJP). Figure 3 depicts a CT image and VATS lung biopsy of a case of Birt–Hogg–Dube (BHD) syndrome. Figure 4 shows CT and bronchoscopy image of a patient of Sjogren’s with CLD. Figure 5 depicts a CT image of a patient of LAM presenting with pneumothorax. Figure 6 depicts CT images of a LAM patient.

Figs 2A and B: (Patient no. 2) – (A) Coronal section of CT chest showing diffuse cysts and ground glassing (red arrow); (B) PJP cysts seen on GMS stain (white arrow)

Figs 3A to D: (Patient no. 3) – (A) Coronal and axial CT images showing characteristic thin-walled cysts (yellow arrow) and loculated pneumothorax left upper lobe (red arrow); (B) Axial CT images post-VATS resection of pneumothorax; (C) Hematoxylin and eosin stain (400× magnification) of VATS resected specimen showing the cyst wall lined by plump type 2 pneumocytes with underlying loose fibrocollagenous stroma; (D) Hematoxylin and eosin stain (40× magnification) cystic change of the lung showing alveoli in alveoli pattern

Figs 4A and B: (Patient no. 5) – (A) Axial and coronal section of CT chest showing diffuse unilateral cyst (red arrow); (B) Bronchoscopy revealed engorged, widened hyperemic secondary carina (black arrow)

Figs 5A to C: (Patient no. 6) – (A, B) Represent coronal section of CT chest showing diffuse cysts and pneumothorax left side (red arrow); and (C) Axial section of CT chest depicting pneumothorax and diffuse cysts (yellow arrow)

Figs 6A and B: (Patient no. 10) Axial section of CT chest depicting bilateral diffuse cysts in a case of LAM (red arrow)


Pulmonary cystic diseases are rare orphan diseases. The term CLD refers to diverse pulmonary disorders grouped together, which are characterized by findings of parenchymal cysts on lung imaging, usually more than five cysts.6 Cystic lung disease is not so common among asymptomatic individuals who are below 55 years of age; however, their increasing trend of prevalence has been noticed in literature, with advanced age.2 The peak incidence in age varies according to diseases, but commonly, it is seen in the third or fourth decade of life. Sex-based predilection depends on the underlying disease, e.g., LAM almost exclusively affects females, while pulmonary Langerhans cell histiocytosis (PLCH) most commonly affects young adults with a sex distribution equal to slightly female predominant. No particular gender predilection for BHD. Nearly universal association of PLCH with current or former cigarette smoking is well established. Ennis et al. commented that most of the respiratory physicians in their clinical practice will not come across more than a single CLD case per year.7,8 In our study, we included a total of 14 cases seen by different respiratory physicians over a period of two-and-a-half years. The age and gender-based distribution in our study was in agreement with previous reported incidences. The higher number of patients could be attributed to the center being a tertiary care referral center.

Cystic lung disease in lung parenchyma may be caused by various rare disorders of the lungs. The diseases mainly associated with CLD are LAM, PLCH, folliculin gene-associated syndrome (FLCN-S) or BHD syndrome, lymphocytic interstitial pneumonia (LIP), PJP, pulmonary amyloidosis, light-chain deposition disease (LCDD), and metastases of sarcomas. Other rare causes of CLD include desquamative interstitial pneumonia (DIP), hypersensitivity pneumonitis (HP), follicular and constrictive bronchiolitis, hereditary syndromes like Marfan syndrome, neurofibromatosis 1, Ehlers–Danlos syndrome, Proteus syndrome, infectious causes, e.g., chronic pulmonary paracoccidioidomycosis and coccidioidomycosis and tracheobronchial papillomatosis.8,9 The cyst formation pathogenesis remains unclear and several mechanisms have been suggested in different diseases for cyst formation. Distal over inflation after a check valve obstruction is one of the common known mechanisms causing lung cysts and this mechanism has been linked with disease such as foreign body, pneumatocele, metastatic neoplasm, PLCH, and LAM.1012 Another pathway thought to be inducing cyst formation in lungs is ischemia. Obstruction of small capillaries that supply terminal bronchiole causes airway ischemic necrosis and consequent dilatation.13 A molecular mechanism representing LAM, LCH, and LCDD has also been proposed in which various enzymes, e.g., matrix metalloproteinase (MMP), podoplanin (D2-40), and matrix-degradation enzymes, induce remodeling of lung tissue.14 Genetic mutation also plays a role in pathogenesis of CLD. Birt–Hogg–Dubé syndrome is one such example where the lung cyst is usually found abutting interlobular septa with no inflammation. Here, the abnormality is suggested at the alveolar–septal junction. Loss-of-function mutations on chromosome 17p in the folliculin gene (FLCN) cause BHD.15

Regardless of the cause of the cyst, the clinical presentation of patients is often asymptomatic with incidental detection of cysts on chest imaging for some other reason. They can also present with cough and shortness of breath. In cases of spontaneous pneumothorax, the patient may present with acute shortness of breath. In our study, most of the cases presented for evaluation of breathlessness and cough. Pneumothorax has been identified as a sentinel event in CLD. In our study, four patients had pneumothorax as the first presentation that assisted in diagnosis. A careful history about previous pneumothoraces and exposure, e.g., smoking, vaping, and drug use should be sought. Thorough past medical history, family, and occupational history, including previous skin and kidney disorders, hematological disorders and malignancy are of importance. Two cases of PJP in our study were HIV positive, while a case of metastatic cyst was a known case of colorectal cancer. Such clues emphasize the importance of past history in reaching a diagnosis and further management of these cases. If tuberous sclerosis complex (TSC) is suspected, then family history of renal disease, epilepsy, or learning difficulties is useful. Since new mutations are common, a negative family history does not exclude the diagnosis.

Definitive diagnosis may require bronchoalveolar lavage (BAL) and biopsy for histopathological confirmation depending on the underlying disease suspicion. Certain serological tests can be helpful in establishing the diagnosis of specific lung diseases. For example, in suspected cases of systemic connective tissue disorder that might be associated with LIP, especially Sjogren syndrome, serologic studies such as antinuclear antibody, anti-Ro/SSA, and anti-La/SSB antibodies, and a rheumatoid factor should be obtained. In common variable immunodeficiency suspects, serum immunoglobulin levels and HIV need to be tested. VEGF-D levels in serum are elevated in many patients with sporadic and TSC LAM. A level of VEGF-D above 800 pg/mL is highly specific for LAM and, in a patient with compatible radiographic features, is considered diagnostic and invasive modalities are not recommended.16,17 FLCN mutation analysis is available as a diagnostic test and detects mutations in 81–88% of patients with BHD.18 Because of the complexity of understanding of these diseases and the requirement for specialized investigations, reaching a final diagnosis may take some time.7,19

Radiological assessment of pulmonary cysts primarily needs differentiation from pulmonary cavities because of very different etiologies of these two entities. Careful observation of cyst size and shape, as well as the presence of any ancillary findings, also helps in refining differential diagnosis. Ultimately, histopathology correlation is required in many cases. High-resolution computed tomography appearances of LCH in early stages may present as nodules, while cysts tend to develop later, predominantly distributed diffusely mainly in the upper lobes. The cysts in LAM are thin-walled and round, which usually spares the extreme apices and may involve juxtaphrenic recess. In LIP, universal features are ground-glass opacities and nodules, small cysts (less than 3 cm), which are thin-walled and distributed in random distribution. Identification of small cysts dispersed within the ground-glass opacity is a unique feature of DIP, seen in about a third of patients. In BHD, reports suggest lower zone preponderance for cysts, with a history of recurrent pneumothorax being an important clue.20,21 Lung function test findings depend upon the underlying disease. In BHD, the lung function is usually preserved with reduced diffusion capacity of lung for carbon monoxide (DLCO), while in LAM and PLCH spirometry, findings of obstruction with reduced DLCO are observed. While LIP, amyloidosis, LCDD, etc., are associated with a restriction on spirometry with reduced DLCO.22

A comprehensive review of radiologic abnormality, clinical assessment, and laboratory findings pointing toward either pulmonary or systemic disease may be helpful to distinguish from numerous differentials to arrive at a diagnosis. The treatment of diffuse CLD depends on etiology identification. Various modalities of disease-specific treatments available include mTOR inhibitor Sirolimus in LAM, corticosteroids and chemotherapeutic agents in LCH and LIP, antibiotics and corticosteroids in Pneumocystis pneumonia infection, treatment of underlying infection, malignancy and lymphoproliferative disorders and lung transplantation in many advanced diseases.21,23 Cooley et al. have elucidated in their study that since CLD-associated spontaneous pneumothorax has a very high rate of recurrence and therefore pleurodesis should be offered to these patients after the very first episode.24 Also, pleurodesis is not a contraindication for lung transplant in these patients. Another technique, total pleural covering (TPC), which involves wrapping the visceral pleura in a bioabsorbable mesh, has reported favorable outcomes in preventing pneumothorax recurrence in CLD.25


The retrospective single-center design and small cohort size limit the generalizability of our findings. Moreover, patient selection bias as the hospital is a tertiary care referral center. Also, spirometry and biopsy were not available for all patients.


Cystic lung disease, though rare, is not infrequent, and most pulmonologists encounter these cases. Due to lack of detailed reliable information about such conditions, patients as well as treating physicians may feel isolated and lonely about CLD. Awareness about CLD and a multidisciplinary approach can assist in identifying the etiology. With the availability of serological tests (like VEGF), biopsy is not mandatory in all cases. Due to myriad presentations and differentials, a holistic insight is required. Detailed history, regular follow-up, pleurodesis, and counseling can impact the patient’s morbidity and mortality. Forthcoming decades will improve upon the prevailing knowledge of such diseases and enable us to better evaluate and prescribe personalized treatment. It is also important to have a national registry for this group of disorders.


Priyanka Singh https://orcid.org/0000-0002-2468-8394

Amit Singh Wasan https://orcid.org/0000-0002-6698-0449


1. Araki T, Nishino M, Gao W, et al. Pulmonary cysts identified on chest CT: Are they part of aging change or of clinical significance? Thorax 2015;70(12):1156–1162. DOI: 10.1136/thoraxjnl-2015-207653.

2. Copley SJ, Wells AU, Hawtin KE, et al. Lung morphology in the elderly: Comparative CT study of subjects over 75 years old versus those under 55 years old. Radiology 2009;251(2):566–573. DOI: 10.1148/radiol.2512081242.

3. Baldi BG, Carvalho CRR, Dias OM, et al. Diffuse cystic lung diseases: Differential diagnosis. J Bras Pneumol 2017;43(2):140–149. DOI: 10.1590/S1806-37562016000000341.

4. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung disease. Part I. Am J Respir Crit Care Med 2015;191(12):1354–1366. DOI: 10.1164/rccm.201411-2094CI.

5. Kwon YS, Han J, Jung KH, et al. Mycobacterium avium lung disease combined with a bronchogenic cyst in an immunocompetent young adult. Korean J Intern Med 2013;28(1):94–97. DOI: 10.3904/kjim.2013.28.1.94.

6. Park S, Lee EJ. Diagnosis and treatment of cystic lung disease. Korean J Intern Med 2017;32(2):229–238. DOI: 10.3904/kjim.2016.242.

7. Ennis S, Silverstone EJ, Yates DH. Investigating cystic lung disease: A respiratory detective approach. Breathe 2020;16(2). Available from: https://breathe.ersjournals.com/content/16/2/200041.

8. Francisco FAF, Souza AS, Zanetti G, et al. Multiple cystic lung disease. Eur Respir Rev 2015;24(138):552–564. DOI: 10.1164/rccm.201411-2094CI. PMID: 25906089.

9. Cosgrove GP, Frankel SK, Brown KK. Challenges in pulmonary fibrosis 3: Cystic lung disease. Thorax 2007;62(9):820–829. DOI: 10.1136/thx.2004.031013.

10. Clarke BE. Cystic lung disease. J Clin Pathol 2013;66(10):904–908. DOI: 10.1136/jclinpath-2012-201297.

11. Dines DE. Diagnostic significance of pneumatocele of the lung. JAMA 1968 24; 204(13):1169–1172.PMID: 5694694.

12. Jeon SY, Yhim HY, Lee NR. Epithelioid sarcoma with spontaneous pneumothorax and massive pleural effusion. Korean J Intern Med 2016;31(1):191–193. DOI: 10.3904/kjim.2016.31.1.191.

13. Gupta N, Vassallo R, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung disease. Part II. Am J Respir Crit Care Med 2015;192(1):17–29. DOI: 10.1164/rccm.201411-2096CI.

14. Colombat M, Caudroy S, Lagonotte E, et al. Pathomechanisms of cyst formation in pulmonary light chain deposition disease. Eur Respir J 2008;32(5):1399–1403. DOI: 10.1183/09031936.00132007.

15. Kennedy JC, Khabibullin D, Henske EP. Mechanisms of pulmonary cyst pathogenesis in Birt-Hogg-Dube syndrome: The stretch hypothesis. Semin Cell Dev Biol 2016;52:47–52. DOI: 10.1016/j.semcdb.2016.02.014.

16. McCormack FX, Gupta N, Finlay GR, et al. Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis diagnosis and management. Am J Respir Crit Care Med 2016;194(6):748–761. DOI: 10.1164/rccm.201607-1384ST.

17. Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis diagnosis and management: High-resolution chest computed tomography, transbronchial lung biopsy, and pleural disease management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline. Am J Respir Crit Care Med 2017;196(10):1337–1348. DOI: 10.1164/rccm.201709-1965ST.

18. Toro JR, Wei MH, Glenn GM, et al. BHD mutations, clinical and molecular genetic investigations of Birt-Hogg-Dubé syndrome: A new series of 50 families and a review of published reports. J Med Genet 2008;45(6):321–331. DOI: 10.1136/jmg.2007.054304.

19. Raoof S, Bondalapati P, Vydyula R, et al. Cystic lung diseases: Algorithmic approach. Chest 2016;150(4):945–965. DOI: 10.1016/j.chest.2016.04.026.

20. Lee KC, Kang EY, Yong HS, et al. A stepwise diagnostic approach to cystic lung diseases for radiologists. Korean J Radiol 2019;20(9):1368–1380. DOI: 10.3348/kjr.2019.0057.

21. Beddy P, Babar J, Devaraj A. A practical approach to cystic lung disease on HRCT. Insights Imaging 2011;2(1):1–7. DOI: 10.1007/s13244-010-0050-7.

22. Obaidat B, Yazdani D, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung diseases. Respir Care 2020;65(1):111–126. DOI: 10.4187/respcare.07117.

23. Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant Off Publ Int Soc Heart Transplant 2015;34(1):1–15. DOI: 10.1016/j.healun.2014.06.014.

24. Cooley J, Lee YG, Gupta N. Spontaneous pneumothorax in diffuse cystic lung diseases. Curr Opin Pulmonary Med 2017;23(4):323–333. DOI: 10.1097/MCP.0000000000000391.

25. Kurihara M, Mizobuchi T, Kataoka H, et al. A total pleural covering for lymphangioleiomyomatosis prevents pneumothorax recurrence. PLoS One 2016;11(9):e0163637. DOI: 10.1371/journal.pone.0163637.

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