Clinical observation of fatal bilateral spontaneous pneumothorax in wegener granulomatosis, which simulated lung cancer

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Abstract

We present a clinical observation of a 77 year-old patient admitted to the hospital with a sharp deterioration in the course of chronic obstructive pulmonary disease. The results of computed tomography of the chest read in favor of a newly detected malignant neoplasm of the upper lobe of the right lung with invasion into the mediastinum and secondary disseminations in the lower lobe of the left lung and liver. The performed fiber-optic bronchoscopy with a transbronchial lung biopsy did not verify the cancer diagnosis. The patient developed a bilateral spontaneous pneumothorax with the formation of bilateral bronchial-pleural fistulae with a massive air discharge through the pleural drainage. The presence of bilateral large bronchopleural fistulae did not allow a surgical intervention which required a separate intubation of the main bronchi. Minimally invasive techniques were ineffective. The patient died on the third day from the moment of the bilateral pneumothorax development due to severe respiratory failure. The autopsy established the diagnosis of Wegener’s granulomatosis affecting the lungs and kidneys.

About the authors

N. B. Khaydukova

Federal Research Clinical Center of Specialized Medical Care and Medical Technologies of the FMBA of Russia

Author for correspondence.
Email: hirurgessa@mail.ru

врач торакальный хирург отделения хирургии

Russian Federation, Moscow

Yu. A. Khabarov

Federal Research Clinical Center of Specialized Medical Care and Medical Technologies of the FMBA of Russia

Email: dr.khabarov@mail.ru

к.м.н., врач торакальный хирург отделения хирургии

Russian Federation, Moscow

V. A. Stepanov

Federal Research Clinical Center of Specialized Medical Care and Medical Technologies of the FMBA of Russia

Email: zvezdkina@yandex.ru

к.м.н., врач-рентгенолог отделения рентгенологии с кабинетами магнитно-резонансной томографии

Russian Federation, Moscow

E. A. Zvyezdkhina

Federal Research Clinical Center of Specialized Medical Care and Medical Technologies of the FMBA of Russia

Email: zvezdkina@yandex.ru

врач патологоанатомического отделения

Russian Federation, Moscow

Yu. V. Ivanov

Federal Research Clinical Center of Specialized Medical Care and Medical Technologies of the FMBA of Russia

Email: ivanovkb83@yandex.ru

д.м.н., профессор, заслуженный врач РФ, заведующий отделением хирургии

Russian Federation, Moscow

References

  1. Бекетова Т.В. Асимптомное течение поражения легких при гранулематозе с полиангиитом (Вегенера) // Научно-практическая ревматология. 2014. № 52 (1). С. 102–104.
  2. Болдарева Н.С., Злобина Т.И., Антипова О.В. и др. Поражение легких при гранулематозе Вегенера // Современные проблемы ревматологии. 2005. № 3. С. 19–122.
  3. Новиков П.И. Клиническая оценка вариантов течения и прогноза гранулематоза с полиангиитом (Вегенера): Автореф. дисс. … канд. мед. наук. М., 2015.
  4. Семенкова Е.Н., Кривошеев О.Г., Новиков П.И., Осипенко В.И. Поражение легких при гранулематозе Вегенера // Клиническая медицина. 2011. № 1. С. 10–13.
  5. Abdou N.I., Kullman G.J., Hoffman G.S. et al. Wegener’s granulomatosis: Survey of 701 patients in North America. Changes in outcome in the 1990s // The Journal of Rheumatology. 2002. Vol. 29. No. 2. P. 309–316.
  6. Belhassen-Garcia M., Velasco-Tirado V., Alvela-Suaréz L. et al. Spontaneous pneumothorax in Wegener’s granulomatosis: Case report and literature review // The Seminars in Arthritis and Rheumatism. 2011. Vol. 41. No. 3. P. 455–460.
  7. Bulbul Y., Ozlu T., Oztuna F. Wegener’s granulomatosis with parotid gland involvement and pneumothorax // Medical Principles and Practice. 2003. Vol. 12. P. 133–137.
  8. Epstein D.M., Gefter W.B., Miller W.T. et al. Spontaneous pneumothorax: An uncommon manifestation of Wegener granulomatosis // Radiology. 1980. Vol. 135. No. 2. P. 327–328. doi: 10.1148/radiology.135.2.7367621.
  9. Isabelle D., Khellaf M., Andre´ M. et al. Spontaneous pneumothorax in Wegener granulomatosis // CHEST. 2005. Vol. 128. P. 3074–3075.
  10. Jaspan T., Davison A.M., Walker W.C. Spontaneous pneumothorax in Wegener’s granulomatosis // Thorax. 1982. Vol. 37. P. 774–775.
  11. Kahraman Н., Inci М.F., Tokur M., Cetin G.Y. Spontaneous pneumothorax in a patient with granulomatosis with polyangiitis // BMJ Case Reports. 2012. Vol. 52. P. 37–41.
  12. Maguire R., Fauci A.S., Doppman J.L., Wolff S.M. Unusual radiographic features of Wegener’s granulomatosis // American Journal of Roentgenology. 1978. Vol. 130. No. 2. P. 233–238. doi: 10.2214/ajr.130.2.233.
  13. Michel J., Courthaliac C., Andre M. et al. Quid? Pneumothorax complicating Wegener disease with rupture of pleura of cavitary nodule // Journal de Radiologie. 2001. Vol. 82. No. 1. P. 73–75.
  14. Ogawa M., Azemoto R., Makino Y. et al. Pneumothorax in a patient with Wegener’s granulomatosis during treatment with immunosuppressive agents // Journal of Internal Medicine. 1991. Vol. 229. P. 189–192.
  15. Sezer I., Kocabas H., Melikoglu M.A. et al. Spontaneous pneumothorax in Wegener’s granulomatosis: A case report // Modern Rheumatology. 2008. Vol. 18. No. 1. P. 76–80. doi: 10.3109/s10165-007-0007-y.
  16. Shi X., Zhang Y., Lu Y. Risk factors and treatment of pneumothorax secondary to granulomatosis with polyangiitis: A clinical analysis of 25 cases // Journal of Cardiothoracic Surgery. 2018. Vol. 13. P. 7.
  17. Tarabishy A.B., Schulte M., Papaliodis G.N. et al. Wegener’s granulomatosis: Clinical manifestations, differential diagnosis, and management of ocular and systemic disease // Survey of Ophthalmology. 2010. Vol. 55. P. 429–440.
  18. Wolffenbuttel B.H., Weber R.F., Kho G.S. Pyopneumothorax: A rare complication of Wegener’s granulomatosis // European Journal of Respiratory Diseases. 1985. Vol. 67. P. 223–227.
  19. Zhiren Li X.B., Zhen J. The rare pulmonary manifestations of Wegener’s granulomatosis (report of 2 cases and literature review) // Chinese Journal of Practical Internal Medicine. 1992. No. 4. P. 216–217.

Supplementary files

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2. Fig. 1. CT scan of the chest with intravenous contrast at admission. A: a soft tissue formation (marker) is visualized in the upper lobe on the right; B: the formation of moderately accumulates a contrast agent within 10HU (marker), extends to the mediastinal fiber and is adjacent to the superior vena cava. B: In the lower lobe on the left there is another soft-tissue formation (triangular arrow). In addition, multiple paraseptal bulls (circled area) are visualized in the lungs on both sides.

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3. Fig. 2. Radiograph of the chest from 02/10/2017, the Small apical pneumothorax on the right

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4. Fig. 3. CT scan of the chest from 11.12.2017, the state after drainage of the left pleural cavity. A: in the left pleural cavity, in the soft tissues of the chest, free air is visualized in the mediastinum (black arrows); B: compression changes in the lower lobe on the left, a small amount of fluid in the pleural cavities (white arrow)

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5. Fig. 4. CT scan of the chest after 13 days after admission, the state after drainage of the right and left pleural cavities. A: in the pleural cavities, in the soft tissues of the chest, free air remains in the mediastinum, on the right the air volume has increased, on the left it has decreased significantly, the left pneumothorax is slightly expressed in the basal regions (black arrows); B: small amounts of fluid in the pleural cavities and compression changes in the basal regions of both lungs are preserved (white arrow)

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6. Fig. 5. Necrotic node in the lung (right), with a wide band of fibrosis around the periphery (middle) and granulomatosis (left). Stained with hematoxylin-eosin, SW. x 10

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7. Fig. 6. Panvasculitis in the lung with granulomatosis in the peripheral sections. Stained with hematoxylin-eosin, SW. x 20

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8. Fig. 7. Chronic focal focal glomerulonephritis with a typical onset of epithelial crescent formation. Coloring with hematoxylin-eosin, HC. x 20

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Copyright (c) 2018 Khaydukova N.B., Khabarov Y.A., Stepanov V.A., Zvyezdkhina E.A., Ivanov Y.V.

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