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Imaging aspects of the diagnosis of sarcoidosis

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Abstract

Sarcoidosis is a systemic granulomatous disorder of unknown aetiology with a wide spectrum of radiological appearances and almost invariably pulmonary involvement. Lung involvement accounts for most of the morbidity and much of the mortality associated with sarcoidosis. Imaging contributes significantly to the diagnosis and management of patients with sarcoidosis. In typical cases, chest radiography may be sufficient to establish the diagnosis with little margin of error and CT is not necessary. However, CT can play a critical role in several clinical settings: atypical clinical and/or radiographic findings; normal or near-normal chest radiograph but clinical suspicion of sarcoidosis; and detection of complications. Moreover, in many patients, CT findings are atypical and unfamiliar to most radiologists (e.g. sarcoidosis mimicking other lung diseases and vice versa), and in these cases histological confirmation of the diagnosis is recommended. CT is also useful in assessing disease extent and may help to discriminate between reversible and irreversible lung disease, thus providing critical prognostic information. This review concentrates on the more difficult imaging aspects of sarcoidosis, in particular differential diagnosis and disease complications.

Key points

Sarcoidosis is characterized by a wide spectrum of radiological appearances.

In typical cases, imaging substantially contributes to the diagnosis of sarcoidosis.

CT plays a critical role in atypical and complicated cases.

CT may discriminate between reversible and irreversible lung disease.

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References

  1. (1999) Statement on sarcoidosis. Joint statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 160:736–755

  2. Baughman RP, Teirstein AS, Judson MA et al (2001) Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 164:1885–1889

    Article  CAS  PubMed  Google Scholar 

  3. Baughman RP, Culver DA, Judson MA (2011) A concise review of pulmonary sarcoidosis. Am J Respir Crit Care Med 183:573–581

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  4. Lynch JP 3rd, Ma YL, Koss MN, White ES (2007) Pulmonary sarcoidosis. Semin Respir Crit Care Med 28:53–74

    Article  PubMed  Google Scholar 

  5. Scadding JG (1961) Prognosis of intrathoracic sarcoidosis in England: a review of 136 cases after five years’ observation. BMJ 2:1165–1172

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  6. Mañá J, Gómez-Vaquero C, Montero A et al (1999) Löfgren’s syndrome revisited: a study of 186 patients. Am J Med 107:240–245

    Article  PubMed  Google Scholar 

  7. Spagnolo P, Grunewald J (2013) Recent advances in the genetics of sarcoidosis. J Med Genet 50:290–297

    Article  CAS  PubMed  Google Scholar 

  8. Mana J, Teirstein AS, Mendelson DS, Padilla ML, DePalo LR (1995) Excessive thoracic computed tomographic scanning in sarcoidosis. Thorax 50:1264–1266

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Remy-Jardin M, Giraud F, Remy J, Wattinne L, Wallaert B, Duhamel A (1994) Pulmonary sarcoidosis: role of CT in the evaluation of disease activity and functional impairment and in prognosis assessment. Radiology 191:675–680

    CAS  PubMed  Google Scholar 

  10. Leung AN, Brauner MW, Caillat-Vigneron N, Valeyre D, Grenier P (1998) Sarcoidosis activity: correlation of HRCT findings with those of 67Ga scanning, bronchoalveolar lavage, and serum angiotensin-converting enzyme assay. J Comput Assist Tomogr 22:229–234

    Article  CAS  PubMed  Google Scholar 

  11. Nishino M, Lee KS, Itoh H, Hatabu H (2010) The spectrum of pulmonary sarcoidosis: variations of high-resolution CT findings and clues for specific diagnosis. Eur J Radiol 73:66–73

    Article  PubMed  Google Scholar 

  12. Criado E, Sanchez M, Ramirez J et al (2010) Pulmonary sarcoidosis: typical and atypical manifestations at high-resolution CT with pathologic correlation. Radiographics 30:1567–1586

    Article  PubMed  Google Scholar 

  13. Brauner MW, Grenier P, Mompoint D, Lenoir S, de Crémoux H (1989) Pulmonary sarcoidosis: evaluation with high-resolution CT. Radiology 172:467–471

    CAS  PubMed  Google Scholar 

  14. Müller NL, Kullnig P, Miller RR (1989) The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J Roentgen 152:1179–1182

    Article  Google Scholar 

  15. Raoof S, Amchentsev A, Vlahos I, Goud A, Naidich DP (2006) Pictorial essay: multinodular disease: a high-resolution CT scan diagnostic algorithm. Chest 129:805–815

    Article  PubMed  Google Scholar 

  16. Nunes H, Uzunhan Y, Gille T, Lamberto C, Valeyre D, Brillet PY (2012) Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function. Eur Respir J 40:750–765

    Article  PubMed  Google Scholar 

  17. Hansell DM, Milne DG, Wilsher ML, Wells AU (1998) Pulmonary sarcoidosis: morphologic associations of airflow obstruction at thin-section CT. Radiology 209:697–704

    CAS  PubMed  Google Scholar 

  18. Davies CW, Tasker AD, Padley SP, Davies RJ, Gleeson FV (2000) Air trapping in sarcoidosis on computed tomography: correlation with lung function. Clin Radiol 55:217–221

    Article  CAS  PubMed  Google Scholar 

  19. Terasaki H, Fujimoto K, Müller NL, Sadohara J, Uchida M, Koga T et al (2005) Pulmonary sarcoidosis: comparison of findings of inspiratory and expiratory high-resolution CA and pulmonary function tests between smokers and nonsmokers. AJR Am J Roentgen 185:333–338

    Article  Google Scholar 

  20. Rabinowitz JG, Ulreich S, Soriano C (1974) The usual unusual manifestations of sarcoidosis and the “hilar haze” - a new diagnostic aid. Am J Roentgenol Radium Ther Nucl Med 120:821–831

    Article  CAS  PubMed  Google Scholar 

  21. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu G, MacIntyre NR et al (1989) Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology 171:619–624

    CAS  PubMed  Google Scholar 

  22. Akira M, Kozuka T, Inoue Y, Sakatani M (2005) Long-term follow-up CT scan evaluation in patients with pulmonary sarcoidosis. Chest 127:185–191

    Article  PubMed  Google Scholar 

  23. Muller NL, Miller RR (1993) Ground-glass attenuation, nodules, alveolitis, and sarcoid granulomas. Radiology 189:31–32

    CAS  PubMed  Google Scholar 

  24. Winterbauer RH, Belic N, Moores KD (1973) Clinical interpretation of bilateral hilar adenopathy. Ann Intern Med 78:65–71

    Article  CAS  PubMed  Google Scholar 

  25. Gawne-Cain ML, Hansell DM (1996) The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis and tuberculosis: a CT study. Clin Radiol 51:263–267

    Article  CAS  PubMed  Google Scholar 

  26. Trisolini R, Anevlavis S, Tinelli C, Orlandi P, Patelli M (2013) CT pattern of lymphadenopathy in untreated patients undergoing bronchoscopy for suspected sarcoidosis. Respir Med 107:897–903

    Article  PubMed  Google Scholar 

  27. Abehsera M, Valeyre D, Grenier P, Jaillet H, Battesti JP, Brauner MW (2000) Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function. AJR Am J Roentgenol 174:1751–1757

    Article  CAS  PubMed  Google Scholar 

  28. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T (1993) Pulmonary sarcoidosis: correlation of CT and histopathologic findings. Radiology 189:105–109

    CAS  PubMed  Google Scholar 

  29. Honda O, Johkoh T, Ichikado K et al (1999) Comparison of high resolution CT findings of sarcoidosis, lymphoma, and lymphangitic carcinoma: is there any difference of involved interstitium? J Comput Assist Tomogr 23:374–379

    Article  CAS  PubMed  Google Scholar 

  30. Grenier P, Chevret S, Beigelman C, Brauner MW, Chastang C, Valeyre D (1994) Chronic diffuse infiltrative lung disease: determination of the diagnostic value of clinical data, chest radiography, and CT and Bayesian analysis. Radiology 191:383–390

    CAS  PubMed  Google Scholar 

  31. Gross BH, Schneider HJ, Proto AV (1980) Eggshell calcification of lymph nodes: an update. AJR Am J Roentgenol 135:1265–1268

    Article  CAS  PubMed  Google Scholar 

  32. Spann RW, Rosenow EC 3rd, DeRemee RA, Miller WE (1971) Unilateral hilar or paratracheal adenopathy in sarcoidosis: a study of 38 cases. Thorax 26:296–299

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  33. Nakatsu M, Hatabu H, Morikawa K et al (2002) Large coalescent parenchymal nodules in pulmonary sarcoidosis: “sarcoid galaxy” sign. AJR Am J Roentgenol 178:1389–1393

    Article  PubMed  Google Scholar 

  34. Hawtin KE, Roddie ME, Mauri FA, Copley SJ (2010) Pulmonary sarcoidosis: the ‘Great Pretender’. Clin Radiol 65:642–650

    Article  CAS  PubMed  Google Scholar 

  35. Reddy TL, Tominaga M, Hansell DM et al (2012) Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 40:377–385

    Article  PubMed  Google Scholar 

  36. Padley SP, Padhani AR, Nicholson A, Hansell DM (1996) Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT. Clin Radiol 51:807–810

    Article  CAS  PubMed  Google Scholar 

  37. (1997) Primary immunodeficiency diseases: report of a WHO Scientific Group. Clin Exp Immunol 109(Suppl 1):1–28

  38. Cunningham-Rundles C, Bodian C (1999) Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol 92:34–48

    Article  CAS  PubMed  Google Scholar 

  39. Ardeniz O, Cunningham-Rundles C (2009) Granulomatous disease in common variable immunodeficiency. Clin Immunol 133:198–207

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  40. Oksenhendler E, Gérard L, Fieschi C et al (2008) Infections in 252 patients with common variable immunodeficiency. Clin Infect Dis 46:1547–1554

    Article  PubMed  Google Scholar 

  41. Fasano MB, Sullivan KE, Sarpong SB et al (1996) Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature. Medicine 75:251–261

    Article  CAS  PubMed  Google Scholar 

  42. Mechanic LJ, Dikman S, Cunningham-Rundles C (1997) Granulomatous disease in common variable immunodeficiency. Ann Intern Med 127:613–617

    Article  CAS  PubMed  Google Scholar 

  43. Artac H, Bozkurt B, Talim B, Reisli I (2009) Sarcoid-like granulomas in common variable Immunodeficiency. Rheumatology 30:109–112

    CAS  Google Scholar 

  44. Tanaka N, Kim JS, Bates CA et al (2006) Lung diseases in patients with common variable immunodeficiency: chest radiographic, and computed tomographic findings. J Comput Assist Tomogr 30:828–838

    Article  PubMed  Google Scholar 

  45. Park JE, Beal I, Dilworth JP, Tormey V, Haddock J (2005) The HRCT appearances of granulomatous pulmonary disease in common variable immunodeficiency. Eur J Radiol 54:359–364

    Article  CAS  PubMed  Google Scholar 

  46. Bouvry D, Mouthon L, Brillet P-Y et al (2013) Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis. Eur Respir J 41:115–122

    Article  PubMed  Google Scholar 

  47. McLoud TC, Putman CE, Pascual R (1974) Eggshell calcification with systemic sarcoidosis. Chest 66:515–517

    Article  CAS  PubMed  Google Scholar 

  48. Spagnolo P, Luppi F, Roversi P, Cerri S, Fabbri LM, Richeldi L (2012) Sarcoidosis: challenging diagnostic aspect of an old disease. Am J Med 125:118–125

    Article  PubMed  Google Scholar 

  49. Maier LA (2002) Clinical approach to chronic beryllium disease and other nonpneumoconiotic interstitial lung diseases. J Thorac Imaging 17:273–284

    Article  PubMed  Google Scholar 

  50. Newman LS, Buschman DL, Newell JD Jr, Lynch DA (1994) Beryllium disease: assessment with CT. Radiology 190:835–840

    CAS  PubMed  Google Scholar 

  51. Morgan W, Dinman B (1989) Pulmonary effects of aluminum. In: Gitelman HJ (ed) Aluminum and health. Dekker, New York

    Google Scholar 

  52. Kraus T, Schaller KH, Angerer J, Hilgers RD, Letzel S (2006) Aluminosis - detection of an almost forgotten disease with HRCT. J Occup Med Toxicol 1:4

    Article  PubMed Central  PubMed  Google Scholar 

  53. Pena TA, Soubani AO, Samavati L (2011) Aspergillus lung disease in patients with sarcoidosis: a case series and review of the literature. Lung 189:167–172

    Article  PubMed  Google Scholar 

  54. Hours S, Nunes H, Kambouchner M et al (2008) Pulmonary cavitary sarcoidosis: clinico-radiologic characteristics and natural history of a rare form of sarcoidosis. Medicine 87:142–151

    Article  PubMed  Google Scholar 

  55. Israel HL, Ostrow A (1969) Sarcoidosis and aspergilloma. Am J Med 47:243–250

    Article  CAS  PubMed  Google Scholar 

  56. Wollschlager C, Khan F (1984) Aspergillomas complicating sarcoidosis. A prospective study in 100 patients. Chest 86:585–588

    Article  CAS  PubMed  Google Scholar 

  57. Tomlinson JR, Sahn SA (1987) Aspergilloma in sarcoidosis and tuberculosis. Chest 92:505–508

    Article  CAS  PubMed  Google Scholar 

  58. Pesle GD, Monod O (1954) Bronchiectasis due to aspergilloma. Dis Chest 25:172–183

    Article  CAS  PubMed  Google Scholar 

  59. Franquet T, Müller NL, Gimenez A, Guembe P, de La Torre J, Bagué S (2001) Spectrum of pulmonary aspergillosis: histologic, clinical, and radiologic findings. Radiographics 21:825–837

    Article  CAS  PubMed  Google Scholar 

  60. Sansom HE, Baque-Juston M, Wells AU, Hansell DM (2000) Lateral cavity wall thickening as an early radiographic sign of mycetoma formation. Eur Radiol 10:387–390

    Article  CAS  PubMed  Google Scholar 

  61. Nunes H, Humbert M, Capron F et al (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, hemodynamics and prognosis. Thorax 61:68–74

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  62. Corte TJ, Wells AU, Nicholson AG, Hansell DM, Wort SJ (2011) Pulmonary hypertension in sarcoidosis: a review. Respirology 16:69–77

    Article  PubMed  Google Scholar 

  63. Weitzman S, Pocock WA, Hawkins DM, Barlow JB (1974) Observer variation in radiological assessment of pulmonary vasculature. Br J Heart 36:280–290

    Article  CAS  Google Scholar 

  64. Matthay RA, Schwarz MI, Ellis JH et al (1981) Pulmonary artery hypertension in chronic obstructive pulmonary disease: determination by chest radiography. Invest Radiol 16:95–100

    Article  CAS  PubMed  Google Scholar 

  65. Tan RT, Kuzo R, Goodman LR (1998) Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Medical College of Wisconsin Lung Transplant Group. Chest 113:1250–1256

    Article  CAS  PubMed  Google Scholar 

  66. Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM (2008) The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 249:1042–1049

    Article  PubMed  Google Scholar 

  67. Resten A, Maitre S, Humbert M et al (2004) Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol 183:65–70

    Article  PubMed  Google Scholar 

  68. Shorr AF, Davies DB, Nathan SD (2003) Predicting mortality in patients with sarcoidosis awaiting lung transplantation. Chest 124:922–928

    PubMed  Google Scholar 

  69. Arcasoy SM, Christie JD, Pochettino A et al (2001) Characteristics and outcomes of patients with sarcoidosis listed for lung transplantation. Chest 120:873–880

    Article  CAS  PubMed  Google Scholar 

  70. Shorr AF, Davies DB, Nathan SD (2002) Outcome for patients with sarcoidosis awaiting lung transplantation. Chest 122:233–238

    Article  PubMed  Google Scholar 

  71. Gafà G, Sverzellati N, Bonati E et al (2012) Follow-up in pulmonary sarcoidosis: comparison between HRCT and pulmonary function tests. Radiol Med 117:968–978

    Article  PubMed  Google Scholar 

  72. Naccache JM, Lavole A, Nunes H et al (2008) High-resolution computed tomographic imaging of airways in sarcoidosis patients with airflow obstruction. J Comput Assist Tomogr 32:905–912

    Article  PubMed  Google Scholar 

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Spagnolo, P., Sverzellati, N., Wells, A.U. et al. Imaging aspects of the diagnosis of sarcoidosis. Eur Radiol 24, 807–816 (2014). https://doi.org/10.1007/s00330-013-3088-3

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