Skip to main content

Advanced (“End-Stage”) Pulmonary Sarcoidosis

  • Chapter
  • First Online:
Pulmonary Sarcoidosis

Part of the book series: Respiratory Medicine ((RM,volume 17))

Abstract

Sarcoidosis is rarely an “end-stage” disease—a concept that implies futility of further treatment. Instead, we suggest the use of the term advanced pulmonary sarcoidosis which encompasses the ideas that the disease has progressed to the point that there is substantial morbidity, there is relatively high risk of mortality, and less reversibility than usual pulmonary sarcoidosis. While it may be equated with chronic, progressive, or fibrotic disease, none of those phenotypes are synonymous with advanced pulmonary sarcoidosis, since all of them may be asymptomatic or do not require therapy.

Symptoms and functional impairment in advanced pulmonary sarcoidosis may be multifactorial. It is important to define the pathophysiologic mechanism for limitations to direct therapy appropriately. A substantial proportion of patients with advanced pulmonary sarcoidosis have related pathologies, including pulmonary hypertension, bronchiectasis, airways stenosis, and mycetoma. These issues should be sought out and treated specifically. Second, the concept that most advanced pulmonary sarcoidosis is irreversible may lead to under-treatment of fibrotic pulmonary disease. There are no biomarkers of disease activity that are adequate to predict futility of immunosuppressive therapy trials in advanced disease.

When immunosuppressive therapy is no longer effective, lung transplantation may be considered. Lung transplantation for sarcoidosis is as successful as for other indications. Although sarcoidosis may recur in the allograft, it is usually non-bothersome. This chapter will review the definition of advanced pulmonary sarcoidosis, risk factors for it, typical manifestations, and approach to nonspecific and specific therapy for it, including lung transplant as a last option.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

ACCESS:

A Case–Control Etiologic Sarcoidosis Study

ACE:

Angiotensin-converting enzyme

BAE:

Bronchial artery embolization

BAL:

Bronchoalveolar lavage

BOS:

Bronchiolitis obliterans syndrome

COPD:

Chronic obstructive pulmonary disease

CT:

Computed tomography

DLCO:

Diffusing capacity of the lung for carbon monoxide

FDG:

18F-Fluorodeoxyglucose

FEV1:

Forced expiratory volume in 1 s

FVC:

Forced vital capacity

HLA:

Human leukocyte antigen

IL:

Interleukin

IPF:

Idiopathic pulmonary fibrosis

ISHLT:

International Society for Heart and Lung Transplantation

LAS:

Lung Allocation Score

PET:

Positron emission test

PFTs:

Pulmonary function tests

PH:

Pulmonary hypertension

SAPH:

Sarcoidosis-associated pulmonary hypertension

TLC:

Total lung capacity

UIP:

Usual interstitial pneumonitis

VO2max:

Maximal oxygen consumption

WASOG:

World Association of Sarcoidosis and Other Granulomatous Disorders

References

  1. Bellomo R, Ronco C, Kellum JA, Mehta RL, Palevsky P. Acute renal failure – definition, outcome measures, animal models, fluid therapy and information technology needs: the Second International Consensus Conference of the Acute Dialysis Quality Initiative (ADQI) Group. Crit Care. 2004;8(4):R204–12.

    PubMed  Google Scholar 

  2. Baughman RP, Nagai S, Balter M, Costabel U, Drent M, du Bois R, et al. Defining the clinical outcome status (COS) in sarcoidosis: results of WASOG Task Force. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28(1):56–64.

    CAS  PubMed  Google Scholar 

  3. Prasse A, Katic C, Germann M, Buchwald A, Zissel G, Muller-Quernheim J. Phenotyping sarcoidosis from a pulmonary perspective. Am J Respir Crit Care Med. 2008;177(3):330–6.

    CAS  PubMed  Google Scholar 

  4. Nardi A, Brillet PY, Letoumelin P, Girard F, Brauner M, Uzunhan Y, et al. Stage IV sarcoidosis: comparison of survival with the general population and causes of death. Eur Respir J. 2011;38(6):1368–73.

    CAS  PubMed  Google Scholar 

  5. Shigemitsu H, Oblad JM, Sharma OP, Koss MN. Chronic interstitial pneumonitis in end-stage sarcoidosis. Eur Respir J. 2010;35(3):695–7.

    CAS  PubMed  Google Scholar 

  6. Teirstein AT, Morgenthau AS. “End-stage” pulmonary fibrosis in sarcoidosis. Mt Sinai J Med. 2009;76(1):30–6.

    PubMed  Google Scholar 

  7. Xu L, Kligerman S, Burke A. End-stage sarcoid lung disease is distinct from usual interstitial pneumonia. Am J Surg Pathol. 2013;37(4):593–600.

    PubMed  Google Scholar 

  8. Baughman RP, Winget DB, Bowen EH, Lower EE. Predicting respiratory failure in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis. 1997;14(2):154–8.

    CAS  PubMed  Google Scholar 

  9. Judson MA. Lung transplantation for pulmonary sarcoidosis. Eur Respir J. 1998;11(3): 738–44.

    CAS  PubMed  Google Scholar 

  10. Johns CJ, Michele TM. The clinical management of sarcoidosis. A 50-year experience at the Johns Hopkins Hospital. Medicine (Baltimore). 1999;78(2):65–111.

    CAS  Google Scholar 

  11. Nagai S, Shigematsu M, Hamada K, Izumi T. Clinical courses and prognoses of pulmonary sarcoidosis. Curr Opin Pulm Med. 1999;5(5):293–8.

    CAS  PubMed  Google Scholar 

  12. Judson MA, Baughman RP, Thompson BW, Teirstein AS, Terrin ML, Rossman MD, et al. Two year prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vasc Diffuse Lung Dis. 2003;20(3):204–11.

    PubMed  Google Scholar 

  13. Chappell AG, Cheung WY, Hutchings HA. Sarcoidosis: a long-term follow up study. Sarcoidosis Vasc Diffuse Lung Dis. 2000;17(2):167–73.

    CAS  PubMed  Google Scholar 

  14. Mana J, Salazar A, Manresa F. Clinical factors predicting persistence of activity in sarcoidosis: a multivariate analysis of 193 cases. Respiration. 1994;61(4):219–25.

    CAS  PubMed  Google Scholar 

  15. Mana J, Badrinas F. Prognosis of sarcoidosis. An unresolved issue. Sarcoidosis. 1992;9(1): 15–20.

    CAS  PubMed  Google Scholar 

  16. Costabel U, Hunninghake GW. ATS/ERS/WASOG statement on sarcoidosis. Sarcoidosis Statement Committee. American Thoracic Society. European Respiratory Society. World Association for Sarcoidosis and Other Granulomatous Disorders. Eur Respir J. 1999;14(4):735–7.

    CAS  PubMed  Google Scholar 

  17. Akira M, Kozuka T, Inoue Y, Sakatani M. Long-term follow-up CT scan evaluation in patients with pulmonary sarcoidosis. Chest. 2005;127(1):185–91.

    PubMed  Google Scholar 

  18. Judson MA, Boan AD, Lackland DT. The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29(2):119–27.

    CAS  PubMed  Google Scholar 

  19. Swigris JJ, Olson AL, Huie TJ, Fernandez-Perez ER, Solomon J, Sprunger D, et al. Sarcoidosis-related mortality in the United States from 1988 to 2007. Am J Respir Crit Care Med. 2011;183(11):1524–30.

    PubMed  Google Scholar 

  20. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med. 1983;52(208):525–33.

    CAS  PubMed  Google Scholar 

  21. Lazar CA, Culver DA. Treatment of sarcoidosis. Semin Respir Crit Care Med. 2010;31(4):501–18.

    PubMed  Google Scholar 

  22. Scadding JG. Prognosis of intrathoracic sarcoidosis in England. A review of 136 cases after five years’ observation. Br Med J. 1961;2(5261):1165–72.

    CAS  PubMed  Google Scholar 

  23. Hunninghake GW, Gilbert S, Pueringer R, Dayton C, Floerchinger C, Helmers R, et al. Outcome of the treatment for sarcoidosis. Am J Respir Crit Care Med. 1994;149(4 Pt 1):893–8.

    CAS  PubMed  Google Scholar 

  24. Grunewald J. Review: role of genetics in susceptibility and outcome of sarcoidosis. Semin Respir Crit Care Med. 2010;31(4):380–9.

    PubMed  Google Scholar 

  25. Hill MR, Papafili A, Booth H, Lawson P, Hubner M, Beynon H, et al. Functional prostaglandin-endoperoxide synthase 2 polymorphism predicts poor outcome in sarcoidosis. Am J Respir Crit Care Med. 2006;174(8):915–22.

    CAS  PubMed  Google Scholar 

  26. Kruit A, Grutters JC, Ruven HJ, van Moorsel CH, Weiskirchen R, Mengsteab S, et al. Transforming growth factor-beta gene polymorphisms in sarcoidosis patients with and without fibrosis. Chest. 2006;129(6):1584–91.

    CAS  PubMed  Google Scholar 

  27. Yamaguchi E, Itoh A, Hizawa N, Kawakami Y. The gene polymorphism of tumor necrosis factor-beta, but not that of tumor necrosis factor-alpha, is associated with the prognosis of sarcoidosis. Chest. 2001;119(3):753–61.

    CAS  PubMed  Google Scholar 

  28. Lopes AJ, Menezes SL, Dias CM, Oliveira JF, Mainenti MR, Guimaraes FS. Cardiopulmonary exercise testing variables as predictors of long-term outcome in thoracic sarcoidosis. Braz J Med Biol Res. 2012;45(3):256–63.

    CAS  PubMed  Google Scholar 

  29. Vestbo J, Viskum K. Respiratory symptoms at presentation and long-term vital prognosis in patients with pulmonary sarcoidosis. Sarcoidosis. 1994;11(2):123–5.

    CAS  PubMed  Google Scholar 

  30. Reich JM. Mortality of intrathoracic sarcoidosis in referral vs population-based settings: influence of stage, ethnicity, and corticosteroid therapy. Chest. 2002;121(1):32–9.

    PubMed  Google Scholar 

  31. Gribbin J, Hubbard RB, Le Jeune I, Smith CJ, West J, Tata LJ. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax. 2006;61(11):980–5.

    CAS  PubMed  Google Scholar 

  32. Huang CT, Heurich AE, Sutton AL, Lyons HA. Mortality in sarcoidosis. A changing pattern of the causes of death. Eur J Respir Dis. 1981;62(4):231–8.

    CAS  PubMed  Google Scholar 

  33. Iwai K, Tachibana T, Hosoda Y, Matsui Y. Sarcoidosis autopsies in Japan. Frequency and trend in the last 28 years. Sarcoidosis. 1988;5(1):60–5.

    CAS  PubMed  Google Scholar 

  34. Rabin DL, Thompson B, Brown KM, Judson MA, Huang X, Lackland DT, et al. Sarcoidosis: social predictors of severity at presentation. Eur Respir J. 2004;24(4):601–8.

    CAS  PubMed  Google Scholar 

  35. Gideon NM, Mannino DM. Sarcoidosis mortality in the United States 1979-1991: an analysis of multiple-cause mortality data. Am J Med. 1996;100(4):423–7.

    CAS  PubMed  Google Scholar 

  36. Viskum K, Vestbo J. Vital prognosis in intrathoracic sarcoidosis with special reference to pulmonary function and radiological stage. Eur Respir J. 1993;6(3):349–53.

    CAS  PubMed  Google Scholar 

  37. Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis-associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest. 2010;138(5):1078–85.

    PubMed  Google Scholar 

  38. Shlobin OA, Nathan SD. Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation. Eur Respir J. 2012;39(6):1520–33.

    PubMed  Google Scholar 

  39. Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager Jr H, Bresnitz EA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001;164(10 Pt 1):1885–9.

    CAS  PubMed  Google Scholar 

  40. Moller DR. Pulmonary fibrosis of sarcoidosis. New approaches, old ideas. Am J Respir Cell Mol Biol. 2003;29(3 Suppl):S37–41.

    CAS  PubMed  Google Scholar 

  41. Rosen Y. Pathology of sarcoidosis. Semin Respir Crit Care Med. 2007;28(1):36–52.

    PubMed  Google Scholar 

  42. Cavazza A, Harari S, Caminati A, Barbareschi M, Carbonelli C, Spaggiari L, et al. The histology of pulmonary sarcoidosis: a review with particular emphasis on unusual and underrecognized features. Int J Surg Pathol. 2009;17(3):219–30.

    PubMed  Google Scholar 

  43. Rosen Y, Athanassiades TJ, Moon S, Lyons HA. Nongranulomatous interstitial pneumonitis in sarcoidosis. Relationship to development of epithelioid granulomas. Chest. 1978;74(2):122–5.

    CAS  PubMed  Google Scholar 

  44. El-Husseini A, Mahmood MA, Sabry A. Pulmonary sarcoidosis developing fatal interstitial pneumonia-like lesions: a case report and literature review. Intern Emerg Med. 2011;6(5): 479–81.

    PubMed  Google Scholar 

  45. Nobata K, Kasai T, Fujimura M, Mizuguchi M, Nishi K, Ishiura Y, et al. Pulmonary sarcoidosis with usual interstitial pneumonia distributed predominantly in the lower lung fields. Intern Med. 2006;45(6):359–62.

    PubMed  Google Scholar 

  46. Shigemitsu H, Azuma A. Sarcoidosis and interstitial pulmonary fibrosis; two distinct disorders or two ends of the same spectrum. Curr Opin Pulm Med. 2011;17(5):303–7.

    PubMed  Google Scholar 

  47. Perry A, Vuitch F. Causes of death in patients with sarcoidosis. A morphologic study of 38 autopsies with clinicopathologic correlations. Arch Pathol Lab Med. 1995;119(2):167–72.

    CAS  PubMed  Google Scholar 

  48. Abehsera M, Valeyre D, Grenier P, Jaillet H, Battesti JP, Brauner MW. Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function. AJR Am J Roentgenol. 2000;174(6):1751–7.

    CAS  PubMed  Google Scholar 

  49. Prasse A, Pechkovsky DV, Toews GB, Jungraithmayr W, Kollert F, Goldmann T, et al. A vicious circle of alveolar macrophages and fibroblasts perpetuates pulmonary fibrosis via CCL18. Am J Respir Crit Care Med. 2006;173(7):781–92.

    CAS  PubMed  Google Scholar 

  50. Lockstone HE, Sanderson S, Kulakova N, Baban D, Leonard A, Kok WL, et al. Gene set analysis of lung samples provides insight into pathogenesis of progressive, fibrotic pulmonary sarcoidosis. Am J Respir Crit Care Med. 2010;181(12):1367–75.

    CAS  PubMed  Google Scholar 

  51. Chen ES, Greenlee BM, Wills-Karp M, Moller DR. Attenuation of lung inflammation and fibrosis in interferon-gamma-deficient mice after intratracheal bleomycin. Am J Respir Cell Mol Biol. 2001;24(5):545–55.

    CAS  PubMed  Google Scholar 

  52. Miller A, Teirstein AS, Jackler I, Chuang M, Siltzbach LE. Airway function in chronic pulmonary sarcoidosis with fibrosis. Am Rev Respir Dis. 1974;109(2):179–89.

    CAS  PubMed  Google Scholar 

  53. Lewis MM, Mortelliti MP, Yeager Jr H, Tsou E. Clinical bronchiectasis complicating pulmonary sarcoidosis: case series of seven patients. Sarcoidosis Vasc Diffuse Lung Dis. 2002;19(2): 154–9.

    PubMed  Google Scholar 

  54. Lynch 3rd JP, Kazerooni EA, Gay SE. Pulmonary sarcoidosis. Clin Chest Med. 1997;18(4): 755–85.

    PubMed  Google Scholar 

  55. Jewkes J, Kay PH, Paneth M, Citron KM. Pulmonary aspergilloma: analysis of prognosis in relation to haemoptysis and survey of treatment. Thorax. 1983;38(8):572–8.

    CAS  PubMed  Google Scholar 

  56. Remy-Jardin M, Giraud F, Remy J, Wattinne L, Wallaert B, Duhamel A. Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment. Radiology. 1994;191(3):675–80.

    CAS  PubMed  Google Scholar 

  57. Sharma OP, Johnson R. Airway obstruction in sarcoidosis. A study of 123 nonsmoking black American patients with sarcoidosis. Chest. 1988;94(2):343–6.

    CAS  PubMed  Google Scholar 

  58. Medinger AE, Khouri S, Rohatgi PK. Sarcoidosis: the value of exercise testing. Chest. 2001;120(1):93–101.

    CAS  PubMed  Google Scholar 

  59. Keogh BA, Hunninghake GW, Line BR, Crystal RG. The alveolitis of pulmonary sarcoidosis. Evaluation of natural history and alveolitis-dependent changes in lung function. Am Rev Respir Dis. 1983;128(2):256–65.

    CAS  PubMed  Google Scholar 

  60. Boros PW, Enright PL, Quanjer PH, Borsboom GJ, Wesolowski SP, Hyatt RE. Impaired lung compliance and DL, CO but no restrictive ventilatory defect in sarcoidosis. Eur Respir J. 2010;36(6):1315–22.

    CAS  PubMed  Google Scholar 

  61. Lopes AJ, de Menezes SL, Dias CM, de Oliveira JF, Mainenti MR, Guimaraes FS. Comparison between cardiopulmonary exercise testing parameters and computed tomography findings in patients with thoracic sarcoidosis. Lung. 2011;189(5):425–31.

    PubMed  Google Scholar 

  62. Wallaert B, Talleu C, Wemeau-Stervinou L, Duhamel A, Robin S, Aguilaniu B. Reduction of maximal oxygen uptake in sarcoidosis: relationship with disease severity. Respiration. 2011;82(6):501–8.

    CAS  PubMed  Google Scholar 

  63. Sulica R, Teirstein AS, Kakarla S, Nemani N, Behnegar A, Padilla ML. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest. 2005;128(3):1483–9.

    PubMed  Google Scholar 

  64. Nunes H, Humbert M, Capron F, Brauner M, Sitbon O, Battesti JP, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006;61(1):68–74.

    CAS  PubMed  Google Scholar 

  65. Udwadia ZF, Pilling JR, Jenkins PF, Harrison BD. Bronchoscopic and bronchographic findings in 12 patients with sarcoidosis and severe or progressive airways obstruction. Thorax. 1990;45(4):272–5.

    CAS  PubMed  Google Scholar 

  66. Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010;65 Suppl 1:i1–58.

    PubMed  Google Scholar 

  67. Chambellan A, Turbie P, Nunes H, Brauner M, Battesti JP, Valeyre D. Endoluminal stenosis of proximal bronchi in sarcoidosis: bronchoscopy, function, and evolution. Chest. 2005;127(2):472–81.

    PubMed  Google Scholar 

  68. Olsson T, Bjornstad-Pettersen H, Stjernberg NL. Bronchostenosis due to sarcoidosis: a cause of atelectasis and airway obstruction simulating pulmonary neoplasm and chronic obstructive pulmonary disease. Chest. 1979;75(6):663–6.

    CAS  PubMed  Google Scholar 

  69. Lavergne F, Clerici C, Sadoun D, Brauner M, Battesti JP, Valeyre D. Airway obstruction in bronchial sarcoidosis: outcome with treatment. Chest. 1999;116(5):1194–9.

    CAS  PubMed  Google Scholar 

  70. Lenique F, Brauner MW, Grenier P, Battesti JP, Loiseau A, Valeyre D. CT assessment of bronchi in sarcoidosis: endoscopic and pathologic correlations. Radiology. 1995;194(2):419–23.

    CAS  PubMed  Google Scholar 

  71. Mayse ML, Greenheck J, Friedman M, Kovitz KL. Successful bronchoscopic balloon dilation of nonmalignant tracheobronchial obstruction without fluoroscopy. Chest. 2004;126(2):634–7.

    PubMed  Google Scholar 

  72. Teo F, Anantham D, Feller-Kopman D, Ernst A. Bronchoscopic management of sarcoidosis related bronchial stenosis with adjunctive topical mitomycin C. Ann Thorac Surg. 2010;89(6):2005–7.

    PubMed  Google Scholar 

  73. Fouty BW, Pomeranz M, Thigpen TP, Martin RJ. Dilatation of bronchial stenoses due to sarcoidosis using a flexible fiberoptic bronchoscope. Chest. 1994;106(3):677–80.

    CAS  PubMed  Google Scholar 

  74. Rohatgi PK, Schwab LE. Primary acute pulmonary cavitation in sarcoidosis. AJR Am J Roentgenol. 1980;134(6):1199–203.

    CAS  PubMed  Google Scholar 

  75. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol. 1985;144(3):513–28.

    CAS  PubMed  Google Scholar 

  76. Winterbauer RH, Kraemer KG. The infectious complications of sarcoidosis: a current perspective. Arch Intern Med. 1976;136(12):1356–62.

    CAS  PubMed  Google Scholar 

  77. Israel HL, Ostrow A. Sarcoidosis and aspergilloma. Am J Med. 1969;47(2):243–50.

    CAS  PubMed  Google Scholar 

  78. Battaglini JW, Murray GF, Keagy BA, Starek PJ, Wilcox BR. Surgical management of symptomatic pulmonary aspergilloma. Ann Thorac Surg. 1985;39(6):512–6.

    CAS  PubMed  Google Scholar 

  79. Daly RC, Pairolero PC, Piehler JM, Trastek VF, Payne WS, Bernatz PE. Pulmonary aspergilloma. Results of surgical treatment. J Thorac Cardiovasc Surg. 1986;92(6):981–8.

    CAS  PubMed  Google Scholar 

  80. Hours S, Nunes H, Kambouchner M, Uzunhan Y, Brauner MW, Valeyre D, et al. Pulmonary cavitary sarcoidosis: clinico-radiologic characteristics and natural history of a rare form of sarcoidosis. Medicine (Baltimore). 2008;87(3):142–51.

    Google Scholar 

  81. Stevens DA, Kan VL, Judson MA, Morrison VA, Dummer S, Denning DW, et al. Practice guidelines for diseases caused by Aspergillus. Infectious Diseases Society of America. Clin Infect Dis. 2000;30(4):696–709.

    CAS  PubMed  Google Scholar 

  82. Gorske KJ, Fleming RJ. Mycetoma formation in cavitary pulmonary sarcoidosis. Radiology. 1970;95(2):279–85.

    CAS  PubMed  Google Scholar 

  83. Rubinstein I, Baum GL, Rosenthal T. Fungal infections complicating pulmonary sarcoidosis. J Infect Dis. 1985;152(6):1360.

    CAS  PubMed  Google Scholar 

  84. Waldhorn RE, Tsou E, Kerwin DM. Invasive pulmonary aspergillosis associated with aspergilloma in sarcoidosis. South Med J. 1983;76(2):251–3.

    CAS  PubMed  Google Scholar 

  85. Regnard JF, Icard P, Nicolosi M, Spagiarri L, Magdeleinat P, Jauffret B, et al. Aspergilloma: a series of 89 surgical cases. Ann Thorac Surg. 2000;69(3):898–903.

    CAS  PubMed  Google Scholar 

  86. Khan MA, Dar AM, Kawoosa NU, Ahangar AG, Lone GN, Bashir G, et al. Clinical profile and surgical outcome for pulmonary aspergilloma: nine year retrospective observational study in a tertiary care hospital. Int J Surg. 2011;9(3):267–71.

    PubMed  Google Scholar 

  87. Uflacker R, Kaemmerer A, Neves C, Picon PD. Management of massive hemoptysis by bronchial artery embolization. Radiology. 1983;146(3):627–34.

    CAS  PubMed  Google Scholar 

  88. Kalva SP. Bronchial artery embolization. Tech Vasc Interv Radiol. 2009;12(2):130–8.

    PubMed  Google Scholar 

  89. Mossi F, Maroldi R, Battaglia G, Pinotti G, Tassi G. Indicators predictive of success of embolisation: analysis of 88 patients with haemoptysis. Radiol Med. 2003;105(1–2):48–55.

    PubMed  Google Scholar 

  90. Hammerman KJ, Sarosi GA, Tosh FE. Amphotericin B in the treatment of saprophytic forms of pulmonary aspergillosis. Am Rev Respir Dis. 1974;109(1):57–62.

    CAS  PubMed  Google Scholar 

  91. Campbell JH, Winter JH, Richardson MD, Shankland GS, Banham SW. Treatment of pulmonary aspergilloma with itraconazole. Thorax. 1991;46(11):839–41.

    CAS  PubMed  Google Scholar 

  92. De Beule K, De Doncker P, Cauwenbergh G, Koster M, Legendre R, Blatchford N, et al. The treatment of aspergillosis and aspergilloma with itraconazole, clinical results of an open international study (1982-1987). Mycoses. 1988;31(9):476–85.

    PubMed  Google Scholar 

  93. Dar MA, Ahmad M, Weinstein AJ, Mehta AC, Golish JA. Thoracic aspergillosis (Part I). Overview and aspergilloma. Cleve Clin Q. 1984;51(4):615–30.

    CAS  PubMed  Google Scholar 

  94. Jackson M, Flower CD, Shneerson JM. Treatment of symptomatic pulmonary aspergillomas with intracavitary instillation of amphotericin B through an indwelling catheter. Thorax. 1993;48(9):928–30.

    CAS  PubMed  Google Scholar 

  95. Kravitz JN, Steed LL, Judson MA. Intracavitary voriconazole for the treatment of hemoptysis complicating Pseudallescheria angusta pulmonary mycetomas in fibrocystic sarcoidosis. Med Mycol. 2011;49(2):198–201.

    PubMed  Google Scholar 

  96. Wollschlager C, Khan F. Aspergillomas complicating sarcoidosis. A prospective study in 100 patients. Chest. 1984;86(4):585–8.

    CAS  PubMed  Google Scholar 

  97. Hammerman KJ, Christianson CS, Huntington I, Hurst GA, Zelman M, Tosh FE. Spontaneous lysis of aspergillomata. Chest. 1973;64(6):677–9.

    CAS  PubMed  Google Scholar 

  98. Marcellis RG, Lenssen AF, Elfferich MD, De Vries J, Kassim S, Foerster K, et al. Exercise capacity, muscle strength and fatigue in sarcoidosis. Eur Respir J. 2011;38(3):628–34.

    CAS  PubMed  Google Scholar 

  99. Spruit MA, Thomeer MJ, Gosselink R, Troosters T, Kasran A, Debrock AJ, et al. Skeletal muscle weakness in patients with sarcoidosis and its relationship with exercise intolerance and reduced health status. Thorax. 2005;60(1):32–8.

    CAS  PubMed  Google Scholar 

  100. Phillips WT, Benton MJ, Wagner CL, Riley C. The effect of single set resistance training on strength and functional fitness in pulmonary rehabilitation patients. J Cardiopulm Rehabil. 2006;26(5):330–7.

    PubMed  Google Scholar 

  101. Consensus conference: activity of sarcoidosis. Third WASOG meeting, Los Angeles, USA, September 8-11, 1993. Eur Respir J. 1994;7(3):624–7.

    Google Scholar 

  102. Hunninghake GW, Crystal RG. Pulmonary sarcoidosis: a disorder mediated by excess helper T-lymphocyte activity at sites of disease activity. N Engl J Med. 1981;305(8):429–34.

    CAS  PubMed  Google Scholar 

  103. Rossman MD, Newman LS, Baughman RP, Teirstein A, Weinberger SE, Miller Jr W, et al. A double-blinded, randomized, placebo-controlled trial of infliximab in subjects with active pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23(3):201–8.

    PubMed  Google Scholar 

  104. Mostard RL, Voo S, van Kroonenburgh MJ, Verschakelen JA, Wijnen PA, Nelemans PJ, et al. Inflammatory activity assessment by F18 FDG-PET/CT in persistent symptomatic sarcoidosis. Respir Med. 2011;105(12):1917–24.

    CAS  PubMed  Google Scholar 

  105. Keijsers RG, Grutters JC, Thomeer M, Du Bois RM, Van Buul MM, Lavalaye J, et al. Imaging the inflammatory activity of sarcoidosis: sensitivity and inter observer agreement of (67)Ga imaging and (18)F-FDG PET. Q J Nucl Med Mol Imaging. 2011;55(1):66–71.

    CAS  PubMed  Google Scholar 

  106. Keijsers RG, Verzijlbergen JF, van Diepen DM, van den Bosch JM, Grutters JC. 18F-FDG PET in sarcoidosis: an observational study in 12 patients treated with infliximab. Sarcoidosis Vasc Diffuse Lung Dis. 2008;25(2):143–9.

    CAS  PubMed  Google Scholar 

  107. Sekiya M, Ohwada A, Miura K, Takahashi S, Fukuchi Y. Serum vascular endothelial growth factor as a possible prognostic indicator in sarcoidosis. Lung. 2003;181(5):259–65.

    CAS  PubMed  Google Scholar 

  108. Kobayashi J, Kitamura S. Serum KL-6 for the evaluation of active pneumonitis in pulmonary sarcoidosis. Chest. 1996;109(5):1276–82.

    CAS  PubMed  Google Scholar 

  109. Ziegenhagen MW, Rothe ME, Schlaak M, Muller-Quernheim J. Bronchoalveolar and serological parameters reflecting the severity of sarcoidosis. Eur Respir J. 2003;21(3):407–13.

    CAS  PubMed  Google Scholar 

  110. Nathan SD. Lung transplantation: disease-specific considerations for referral. Chest. 2005;127(3):1006–16.

    PubMed  Google Scholar 

  111. Christie JD, Edwards LB, Kucheryavaya AY, Benden C, Dipchand AI, Dobbels F, et al. The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report-2012. J Heart Lung Transplant. 2012;31(10):1073–86.

    PubMed  Google Scholar 

  112. Shorr AF, Helman DL, Davies DB, Nathan SD. Sarcoidosis, race, and short-term outcomes following lung transplantation. Chest. 2004;125(3):990–6.

    PubMed  Google Scholar 

  113. De Meester J, Smits JM, Persijn GG, Haverich A. Listing for lung transplantation: life expectancy and transplant effect, stratified by type of end-stage lung disease, the Eurotransplant experience. J Heart Lung Transplant. 2001;20(5):518–24.

    PubMed  Google Scholar 

  114. Kreider M, Kotloff RM. Selection of candidates for lung transplantation. Proc Am Thorac Soc. 2009;6(1):20–7.

    PubMed  Google Scholar 

  115. Shorr AF, Davies DB, Nathan SD. Predicting mortality in patients with sarcoidosis awaiting lung transplantation. Chest. 2003;124(3):922–8.

    PubMed  Google Scholar 

  116. Arcasoy SM, Christie JD, Pochettino A, Rosengard BR, Blumenthal NP, Bavaria JE, et al. Characteristics and outcomes of patients with sarcoidosis listed for lung transplantation. Chest. 2001;120(3):873–80.

    CAS  PubMed  Google Scholar 

  117. Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ, et al. International guidelines for the selection of lung transplant candidates: 2006 update – a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2006;25(7):745–55.

    PubMed  Google Scholar 

  118. Yazaki Y, Isobe M, Hiroe M, Morimoto S, Hiramitsu S, Nakano T, et al. Prognostic determinants of long-term survival in Japanese patients with cardiac sarcoidosis treated with prednisone. Am J Cardiol. 2001;88(9):1006–10.

    CAS  PubMed  Google Scholar 

  119. Kasimir MT, Seebacher G, Jaksch P, Winkler G, Schmid K, Marta GM, et al. Reverse cardiac remodelling in patients with primary pulmonary hypertension after isolated lung transplantation. Eur J Cardiothorac Surg. 2004;26(4):776–81.

    PubMed  Google Scholar 

  120. Hadjiliadis D, Sporn TA, Perfect JR, Tapson VF, Davis RD, Palmer SM. Outcome of lung transplantation in patients with mycetomas. Chest. 2002;121(1):128–34.

    PubMed  Google Scholar 

  121. Shah L. Lung transplantation in sarcoidosis. Semin Respir Crit Care Med. 2007;28(1):134–40.

    PubMed  Google Scholar 

  122. Nunley DR, Hattler B, Keenan RJ, Iacono AT, Yousem S, Ohori NP, et al. Lung transplantation for end-stage pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 1999;16(1): 93–100.

    CAS  PubMed  Google Scholar 

  123. Wille KM, Gaggar A, Hajari AS, Leon KJ, Barney JB, Smith KH, et al. Bronchiolitis obliterans syndrome and survival following lung transplantation for patients with sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2008;25(2):117–24.

    CAS  PubMed  Google Scholar 

  124. Scott J, Higenbottam T. Transplantation of the lungs and heart and lung for patients with severe pulmonary complications from sarcoidosis. Sarcoidosis. 1990;7(1):9–11.

    CAS  PubMed  Google Scholar 

  125. Milman N, Burton C, Andersen CB, Carlsen J, Iversen M. Lung transplantation for end-stage pulmonary sarcoidosis: outcome in a series of seven consecutive patients. Sarcoidosis Vasc Diffuse Lung Dis. 2005;22(3):222–8.

    PubMed  Google Scholar 

  126. Girgis RE, Tu I, Berry GJ, Reichenspurner H, Valentine VG, Conte JV, et al. Risk factors for the development of obliterative bronchiolitis after lung transplantation. J Heart Lung Transplant. 1996;15(12):1200–8.

    CAS  PubMed  Google Scholar 

  127. Ionescu DN, Hunt JL, Lomago D, Yousem SA. Recurrent sarcoidosis in lung transplant allografts: granulomas are of recipient origin. Diagn Mol Pathol. 2005;14(3):140–5.

    PubMed  Google Scholar 

  128. Padilla ML, Schilero GJ, Teirstein AS. Sarcoidosis and transplantation. Sarcoidosis Vasc Diffuse Lung Dis. 1997;14(1):16–22.

    CAS  PubMed  Google Scholar 

  129. Heyll A, Meckenstock G, Aul C, Sohngen D, Borchard F, Hadding U, et al. Possible transmission of sarcoidosis via allogeneic bone marrow transplantation. Bone Marrow Transplant. 1994;14(1):161–4.

    CAS  PubMed  Google Scholar 

  130. Johnson BA, Duncan SR, Ohori NP, Paradis IL, Yousem SA, Grgurich WF, et al. Recurrence of sarcoidosis in pulmonary allograft recipients. Am Rev Respir Dis. 1993;148(5):1373–7.

    CAS  PubMed  Google Scholar 

  131. Kazerooni EA, Jackson C, Cascade PN. Sarcoidosis: recurrence of primary disease in transplanted lungs. Radiology. 1994;192(2):461–4.

    CAS  PubMed  Google Scholar 

  132. Kiatboonsri C, Resnick SC, Chan KM, Barbers RG, Marboe CC, Khonsary A, et al. The detection of recurrent sarcoidosis by FDG-PET in a lung transplant recipient. West J Med. 1998;168(2):130–2.

    CAS  PubMed  Google Scholar 

  133. Milman N, Andersen CB, Burton CM, Iversen M. Recurrent sarcoid granulomas in a transplanted lung derive from recipient immune cells. Eur Respir J. 2005;26(3):549–52.

    CAS  PubMed  Google Scholar 

  134. Burke M, Stewart S, Ashcroft T, Corbishley C, Bishop P, Kjellstrom C. Biopsy diagnosis of disease recurrence after transplantation (TX) for pulmonary sarcoidosis: a multicentre study. J Heart Lung Transplant. 2001;20(2):154–5.

    PubMed  Google Scholar 

  135. Walker S, Mikhail G, Banner N, Partridge J, Khaghani A, Burke M, et al. Medium term results of lung transplantation for end stage pulmonary sarcoidosis. Thorax. 1998;53(4): 281–4.

    CAS  PubMed  Google Scholar 

  136. Sharma OP. Sarcoidosis: a historical perspective. Clin Dermatol. 2007;25(3):232–41.

    PubMed  Google Scholar 

  137. Shorr AF, Davies DB, Nathan SD. Outcomes for patients with sarcoidosis awaiting lung transplantation. Chest. 2002;122(1):233–8.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Divya C. Patel D.O. .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Patel, D.C., Budev, M., Culver, D.A. (2014). Advanced (“End-Stage”) Pulmonary Sarcoidosis. In: Judson, M. (eds) Pulmonary Sarcoidosis. Respiratory Medicine, vol 17. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4614-8927-6_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-8927-6_5

  • Published:

  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4614-8926-9

  • Online ISBN: 978-1-4614-8927-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics