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VOLUME 65 , ISSUE 1 ( January-March, 2023 ) > List of Articles

Original Article

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

Priyanka Singh, Amit Singh Vasan, Nitin Balram Ahuja, Saikat Bhattacharjee, Manoj Gopal Madakshira, Arun Hegde

Keywords : Cyst, Lymphangioleiomyomatosis, Pneumothorax, Spirometry, Symptoms

Citation Information : Singh P, Vasan AS, Ahuja NB, Bhattacharjee S, Madakshira MG, Hegde A. Clinico-etiological Characteristics of Cystic Lung Disease: A Retrospective Study. Indian J Chest Dis Allied Sci 2023; 65 (1):32-38.

DOI: 10.5005/jp-journals-11007-0060

License: CC BY-NC 4.0

Published Online: 29-06-2023

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

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.


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  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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