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PROGRESSIVE PULMONARY DYSTROPHY AS ONE OF THE VARIANTS OF BULLOUS LUNGS EMPHYSEMA

Abstract

Progressive pulmonary dystrophy («disappearing lung») is a rare disease of unknown etiology. It is characterized by intensive dystrophic pulmonary changes revealed through the transformation of normal lung tissue into huge air bullas taking 1/3 of lungs volume. This disease is characterized by progressive clinical course, predominantly one-sided character of affection, inconsistency between the scope of affection and bareness of clinical presentations. Personal experience of diagnostics and dynamic examination of 5 patients with progressive pulmonary dystrophy during several years was analyzed. The clinical outcome without surgical correction (the operation of the volume reduction, lung transplantation) is fatal in most cases. The work illustrates different methods of radiodiagnostics, ranging from widely-spread routine ones to modern high-technological ones. The results of exclusive development, namely the method of three dimensional volumetry for quantitative estimation of lungs ventilation function at multispiral computer-aided tomography with the use of inspiratory and expiratory test are presented.

About the Authors

Anatoliy V. Lenshin
Far Eastern Scientific Center of Physiology and Pathology of Respiration SB RAMS
Russian Federation


Andrey V. Il'in
Far Eastern Scientific Center of Physiology and Pathology of Respiration SB RAMS
Russian Federation


Andrey N. Odireev
Far Eastern Scientific Center of Physiology and Pathology of Respiration SB RAMS
Russian Federation


Elizaveta G. Shelud'ko
Far Eastern Scientific Center of Physiology and Pathology of Respiration SB RAMS
Russian Federation


References

1. Вишневский А.А, Борисов В.В., Рустамов И.Р. Дифференциальная диагностика полостных образований легких // Клин. мед. 1988. Т.66, №10. С.14-21.

2. Вишневский А.А., Пикунов М.Ю. Хирургическое лечение буллезной эмфиземы легких // Рос. мед. журн. 1997. №5. С.38-42.

3. Кисты и кистоподобные образования легких / П.В.Власов [и др.] // Медиц. визуализация. 2005. №1. С.82-94.

4. Ильин А.В., Перельман Ю.М., Леншин А.В. Метод диагностики нарушений воздухонаполненности легких у больных бронхиальной астмой // Рос. электрон. журн. лучевой диагностики. 2012. Т.2, №2. Приложение. С.221-222.

5. Коновалов В.К., Шойхет Я.Н. Комплексное исследование буллезных полостей легких // Пульмонология. 2002. №3. С.56-59.

6. Леншин А.В., Гребенник А.Г. Исследование регионарной вентиляционной функции лёгких у больных бронхиальной астмой с помощью компьютерной томографии с инспираторно-экспираторным тестом // Бюл. физиол. и патол. дыхания. 2011. Вып.41. С.18-22.

7. Фунлоэр И.С., Жынжыров Б.К., Юсупов Т.К. Спонтанный пневмоторакс как следствие буллезной болезни // Вестн. Кыргызско-Российского славянского университета. 2011. Т.11, №3. С.157-161.

8. Allison S.T. The vanishing lung; report of case of advanced bullous emphysema // Ann. Int. Med. 1942. Vol.17, №1. P.139-148.

9. Barbaini S., Prato F. Five cases of vanishing lung (author's transl) // Radiol. Med. 1977. Vol.63, №4. P.279-288.

10. Barcan F. Radiological aspects of vanishing lung // Rev. Ig. Bacteriol. Virusol. Parazitol. Epidemiol. Pneumoftiziol. Pneumoftiziol. 1983. Vol.32, №3. P.227-43.

11. Brun J., Kofman J. Progressive bullous emphysema and vanishing lung // Gaz. Med. Fr. 1965. Vol.72, №22. P.3789-3803.

12. Burke R.M. Vanishing lungs: a case report of bullous emphysema // Radiology. 1937. Vol.28. P.367-371.

13. De Filippi G., Grassi E., Randaccio M. Angiographic and bronchographic aspects of idiopathic and secondary vanishing lung in childhood // Radiol. Med. 1976. Vol.62, №4. P.248-254.

14. Placental transmogrification of the lung, a histologic variant of giant bullous emphysema. Clinicopathological study of three further cases / M.E.Fidler [et al.] // Am. J. Surg. Pathol. 1995. Vol.19, №5. Р.563-570.

15. Froeschle P., Krishnadas R., Berrisford R. Video-assisted approach combined with the open Brompton technique for intracavitary drainage of giant bullae // ThoracCardiovasc Surg. 2012. Vol.60, №2. P.164-166.

16. Heilmeyer L., Schmid F. Progressive pulmonary dystrophy; Burke's vanishing lung and de Martini's idiopathic pulmonary atrophy // Dtsch. Med. Wochenschr. 1956. Vol.81, №33. P.1293-1297.

17. Bullous lung disease due to marijuana / S.W.Hii [et al.] // Respirology. 2008. Vol.13, №1. P.122-127.

18. Large lung bullae in marijuana smokers / M.K.Johnson [et al.] // Thorax. 2000. Vol.55, №4. P.340-342.

19. Karkhanis V.S., Joshi J.M. Autobullectomy in idiopathic giant bullous lung disease // Indian J. Chest Dis. Allied Sci. 2010. Vol.52, №3. P.159-160.

20. Kitahara M. Progressive giant bulla and vanishing lung // Ryoikibetsu Shokogun Shirizu. 1994. Vol.3. P.496-498.

21. Roentgenologic picture of the so-called vanishing lung / G.Koppány [et al.] // Orv. Hetil. 1972. Vol.113. №6. P.327-329.

22. Lung volume reduction surgery for emphysema and bullous pulmonary emphysema / F.Le Pimpec-Barthes [et al.] // Rev. Pneumol. Clin. 2012. Vol.68, №2. P.131-145.

23. A patient with vanishing lung syndrome and remarkable tolerance to high altitude / A.M.Luks [et al.] // Med. Sci. Sports Exerc. 2007. Vol.39, №11. P.1891-189.

24. Miller A. The vanishing lung syndrome associated with pulmonary sarcoidosis // Br. J. Dis. Chest. 1981. Vol.75, №2. P.209-214.

25. A case of vanishing lung complicated with pneumothorax and improved significantly by surgery / H.Mizutani [et al.] // Nihon Kokyuki Gakkai Zasshi. 2002. Vol.40, №5. P.365-368.

26. Longitudinal changes in hyperinflation parameters and exercise capacity after giant bullous emphysema surgery / R.Neviere [et al.] // J. Thorac. Cardiovasc. Surg. 2006. Vol.132, №5. P.1203-1207.

27. Phan T.D., Lau K.K., Li X. Lung bullae and pulmonary fibrosis associated with marijuana smoking // Australas. Radiol. 2005. Vol.49, №5. P.411-414.

28. The vanishing lung / N.N.Shah [et al.] // CJEM. 2007. Vol.9, №3. P.233-234.

29. Vanishing lung syndrome (giant bullous emphysema): CT findings in 7 patients and a literature review / N.Sharma [et al.] // J. Thorac. Imaging. 2009. Vol.24, №3. Р.227-230.

30. Simek J., Resl M., Král B. Surgery for bullous emphysema // Acta Medica (Hradec Kralove). 1999. Vol.42, №3. P.111-114.

31. Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients / E.J.Stern [et al.] // Am. J. Roentgenol. 1994. Vol.162, №2. P.279-282.

32. A rare association: Castleman's disease and pemphigus vulgaris / B.Uskül [et al.] // Tuberk. Toraks. 2005. Vol.53, №2.P.172-176.

33. The selection of patients who are candidates for lung transplantation / F.Venuta [et al.] // Minerva Chir. 1996. Vol.51, №1-2. P.5-10.

34. Waitches G.M., Stern E.J., Dubinsky T.J. Usefulness of the double-wall sign in detecting pneumothorax in patients with giant bullous emphysema // Am. J. Roentgenol. 2000. Vol.174, №6. P.1765-1768.

35. Yoshikawa T., Misao T., Aoe M. Primary lung cancer arising from the wall of a giant bulla in which positron emission tomography was useful for preoperative diagnosis // Gen. Thorac. Cardiovasc. Surg. 2011. Vol.59. №2.P.137-140.

36. Vishnevskiy A.A, Borisov V.V., Rustamov I.R. Klinicheskaya meditsina 1988; 66(10):14-21.

37. Vishnevskiy A.A., Pikunov M.Yu. Rossiyskiy meditsinskiy zhurnal 1997; 5:38-42.

38. Vlasov P.V., Karmazanovskiy G.G., Sheykh Zh.V., Vilyavin M..Yu. Meditsinskaya vizualizatsiya 2005; 1:82-94.

39. Il'in A.V., Perelman J.M., Lenshin A.V. Rossiyskiy elektronnyy zhurnal luchevoy diagnostiki 2012; 2(Suppl.2):221-222.

40. Konovalov V.K., Shoykhet Ya.N. Pul'monologiya 2002; 3:56-59.

41. Lenshin A.V., Grebennik A.G. Bûlleten' fiziologii i patologii dyhaniyâ 2011; 41:18-22.

42. Funloer I.S., Zhynzhyrov B.K., Yusupov T.K. Vestnik Kyrgyzsko-Rossiyskogo slavyanskogo universiteta 2011; 11(3):157-161.

43. Allison S.T. The vanishing lung; report of case of advanced bullous emphysema. Ann. Int. Med. 1942; 17(1): 139-148.

44. Barbaini S., Prato F. Five cases of vanishing lung (author's transl). Radiol. Med. 1977; 63(4):279-288.

45. Barcan F. Radiological aspects of vanishing lung. Rev. Ig. Bacteriol. Virusol. Parazitol. Epidemiol. Pneumoftiziol. Pneumoftiziol. 1983; 32(3):227-243.

46. Brun J., Kofman J. Progressive bullous emphysema and vanishing lung. Gaz. Med. Fr. 1965; 72(22):3789-3803.

47. Burke R.M. Vanishing lungs: a case report of bullous emphysema. Radiology 1937; 28:367-371.

48. De Filippi G., Grassi E., Randaccio M. Angiographic and bronchographic aspects of idiopathic and secondary vanishing lung in childhood. Radiol. Med. 1976; 62(4):248-254.

49. Fidler M.E., Koomen M., Sebek B., Greco M.A., Rizk C.C., Askin F.B. Placental transmogrification of the lung, a histologic variant of giant bullous emphysema. Clinicopathological study of three further cases. Am. J. Surg. Pathol. 1995; 19(5):563-570.

50. Froeschle P., Krishnadas R., Berrisford R. Video-assisted approach combined with the open Brompton technique for intracavitary drainage of giant bullae. Thorac. Cardiovasc. Surg. 2012; 60(2):164-166.

51. Heilmeyer L., Schmid F. Progressive pulmonary dystrophy; Burke's vanishing lung and de Martini's idiopathic pulmonary atrophy. Dtsch. Med. Wochenschr. 1956; 81(33):1293-1297.

52. Hii S.W., Tam J.D., Thompson B.R., Naughton M.T. Bullous lung disease due to marijuana. Respirology 2008; 13(1):122-127.

53. Johnson M.K., Smith R.P., Morrison D., Laszlo G., White R.J. Large lung bullae in marijuana smokers. Thorax 2000; 55(4):340-342.

54. Karkhanis V.S., Joshi J.M. Autobullectomy in idiopathic giant bullous lung disease. Indian J. Chest Dis. Allied Sci. 2010; 52(3):159-160.

55. Kitahara M. Progressive giant bulla and vanishing lung. Ryoikibetsu Shokogun Shirizu 1994;3:496-498.

56. Koppány G., Kuchár F., Ujhelyi L., Besznyák I. Roentgenologic picture of the so-called vanishing lung. Orv. Hetil. 1972; 113(6):327-329.

57. Le Pimpec-Barthes F., Das Neves-Pereira J.C., Cazes A., Arame A., Grima R., Hubsch J.P., Zukerman C., Hernigou A., Badia A., Bagan P., Delclaux C., Dusser D., Riquet M. Lung volume reduction surgery for emphysema and bullous pulmonary emphysema. Rev. Pneumol. Clin. 2012; 68(2):131-145.

58. Luks A.M., Goss C.H., Schoene R.B., Swenson E.R. A patient with vanishing lung syndrome and remarkable tolerance to high altitude. Med. Sci. Sports Exerc. 2007; 39(11):1891-1895.

59. Miller A. The vanishing lung syndrome associated with pulmonary sarcoidosis. Br. J. Dis. Chest 1981; 75(2):209-214.

60. Mizutani H., Horiba M., Shindoh J., Kimura T., Son M., Wakahara K. A case of vanishing lung complicated with pneumothorax and improved significantly by surgery. Nihon Kokyuki Gakkai Zasshi 2002; 40(5):365-368.

61. Neviere R., Catto M., Bautin N., Robin S., Porte H., Desbordes J., Matran R. Longitudinal changes in hyperinflation parameters and exercise capacity after giant bullous emphysema surgery. J. Thorac. Cardiovasc. Surg. 2006; 132(5):1203-1207.

62. Phan T.D., Lau K.K., Li X. Lung bullae and pulmonary fibrosis associated with marijuana smoking. Australas. Radiol. 2005; 49(5):411-414.

63. Shah N.N., Bhargava R., Ahmed Z., Pandey D.K., Shameem M., Bachh A.A., Dar K.A. The vanishing lung. CJEM 2007; 9(3):233-234.

64. Sharma N, Justaniah A.M., Kanne J.P., Gurney J.W., Mohammed T.L. Vanishing lung syndrome (giant bullous emphysema): CT findings in 7 patients and a literature review. J. Thorac. Imaging 2009; 24(3):227-230.

65. Simek J., Resl M., Král B. Surgery for bullous emphysema. Acta Medica (Hradec Kralove) 1999; 42(3):111-114.

66. Stern E.J., Webb W.R., Weinacker A., Muller N.L. Idiopathic giant bullous emphysema (vanishing lung syndrome): imaging findings in nine patients. Am. J. Roentgenol. 1994; 162(2):279-282.

67. Uskül B., Ertuğrul M., Selvi A., Mihmanli A., Zindanci I., Türker H. A rare association: Castleman's disease and pemphigus vulgaris. Tuberk. Toraks 2005; 53(2):172-176.

68. Venuta F., Rendina E.A., Ciriaco P., De Giacomo T., Della Rocca G., Lena A., Flaishman I., Servignani M., Ricci C. The selection of patients who are candidates for lung transplantation. Minerva Chir. 1996; 51(1-2):5-10.

69. Waitches G.M., Stern E.J., Dubinsky T.J. Usefulness of the double-wall sign in detecting pneumothorax in patients with giant bullous emphysema. Am. J. Roentgenol. 2000; 174(6):1765-1768.

70. Yoshikawa T., Misao T., Aoe M. Primary lung cancer arising from the wall of a giant bulla in which positron emission tomography was useful for preoperative diagnosis. Gen. Thorac. Cardiovasc. Surg. 2011; 59(2):137-140.


Review

For citations:

Lenshin A.V., Il'in A.V., Odireev A.N., Shelud'ko E.G. PROGRESSIVE PULMONARY DYSTROPHY AS ONE OF THE VARIANTS OF BULLOUS LUNGS EMPHYSEMA. Bulletin Physiology and Pathology of Respiration. 2012;(45):104-114.

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