Pulmonary Sarcoidosis pp 19-39 | Cite as
The Pulmonary Manifestations of Sarcoidosis
- 1.5k Downloads
Abstract
The pulmonary manifestation of sarcoidosis has a great variability and is notorious for mimicking many other interstitial lung diseases. Knowledge of pulmonary manifestations is important in diagnosing saroidosis because thoracic involvement is present in over 90 % of patients. In this chapter, classical findings on chest X-ray and HRCT are described as well as multiple uncommon findings in radiology. The most common findings are bilateral hilar lymphadenopathy, reticulonodular pattern, perilymphatic distribution of nodules, and predominant upper- and middle lobe parenchymal abnormalities. Uncommon findings are unilateral lymphadenopathy, reticular pattern, excessive ground glass opacities, pleural disease, solitary mass, and predominant lower lobe parenchymal abnormalities. Furthermore, the appearance of pulmonary hypertension and necrotizing sarcoid granulomatosis will be described. Finally, imaging of pulmonary sarcoidosis with positron emission tomography with 18F-Fluordeoxyglucose (18F-FDG PET) will be discussed.
Keywords
Sarcoidosis Intrathoracic manifestations Lymphadenopathy Parenchymal involvement Pulmonary fibrosis Chest X-ray HRCT PETAbbreviations
- BHL
Bilateral hilar lymphadenopathy
- COP
Cryptogenic organizing pneumonia
- HRCT
High resolution computed tomography
- LIP
Lymphocytic interstitial pneumonia
- NSG
Necrotizing sarcoid angiitis and granulomatosis
- PET
Positron emission tomography
- SPL
Secondary pulmonary lobule
- SURT
Sarcoidosis of the upper respiratory tract
References
- 1.Hillerdal G, Nou E, Osterman K, Schmekel B. Sarcoidosis: epidemiology and prognosis. A 15-year European study. Am Rev Respir Dis. 1984;130(1):29–32.PubMedGoogle Scholar
- 2.Lynch 3rd JP, Kazerooni EA, Gay SE. Pulmonary sarcoidosis. Clin Chest Med. 1997;18(4): 755–8.PubMedCrossRefGoogle Scholar
- 3.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.PubMedCrossRefGoogle Scholar
- 4.Hendrick DJ, Blackwood RA, Black JM. Chest pain in the presentation of sarcoidosis. Br J Dis Chest. 1976;70(3):206–10.PubMedCrossRefGoogle Scholar
- 5.Cappell MS. Endoscopic, radiographic, and manometric findings in dysphagia associated with sarcoid due to extrinsic esophageal compression from subcarinal lymphadenopathy. Am J Gastroenterol. 1995;90(3):489–92.PubMedGoogle Scholar
- 6.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.PubMedCrossRefGoogle Scholar
- 7.Grutters JC, Drent M, Van den Bosch JMM. European Respiratory Society Monograph 2009; 46(Interstitial Lung Diseases):126-54. doi: 10.1183/1025448x.00046008.
- 8.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. 1999;160(2):736–55.Google Scholar
- 9.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.PubMedCrossRefGoogle Scholar
- 10.Nunes H, Brillet PY, Valeyre D, Brauner MW, Wells AU. Imaging in sarcoidosis. Semin Respir Crit Care Med. 2007;28(1):102–20.PubMedCrossRefGoogle Scholar
- 11.Bechtel JJ, Starr 3rd T, Dantzker DR, Bower JS. Airway hyperreactivity in patients with sarcoidosis. Am Rev Respir Dis. 1981;124(6):759–61.PubMedGoogle Scholar
- 12.Marcias S, Ledda MA, Perra R, Severino C, Rosetti L. Aspecific bronchial hyperreactivity in pulmonary sarcoidosis. Sarcoidosis. 1994;11(2):118–22.PubMedGoogle Scholar
- 13.Shorr AF, Torrington KG, Hnatiuk OW. Endobronchial involvement and airway hyperreactivity in patients with sarcoidosis. Chest. 2001;120(3):881–6.PubMedCrossRefGoogle Scholar
- 14.Baughman RP, Lower EE, Tami T. Upper airway. 4: Sarcoidosis of the upper respiratory tract (SURT). Thorax. 2010;65(2):181–6.PubMedCrossRefGoogle Scholar
- 15.James DG, Barter S, Jash D, MacKinnon DM, Carstairs LS. Sarcoidosis of the upper respiratory tract (SURT). J Laryngol Otol. 1982;96(8):711–8.PubMedCrossRefGoogle Scholar
- 16.Panselinas E, Halstead L, Schlosser RJ, Judson MA. Clinical manifestations, radiographic findings, treatment options, and outcome in sarcoidosis patients with upper respiratory tract involvement. South Med J. 2010;103(9):870–5.PubMedCrossRefGoogle Scholar
- 17.Shorr AF, Torrington KG, Hnatiuk OW. Endobronchial biopsy for sarcoidosis: a prospective study. Chest. 2001;120(1):109–14.PubMedCrossRefGoogle Scholar
- 18.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.PubMedCrossRefGoogle Scholar
- 19.Kirks DR, McCormick VD, Greenspan RH. Pulmonary sarcoidosis. Roentgenologic analysis of 150 patients. Am J Roentgenol Radium Ther Nucl Med. 1973;117(4):777–86.PubMedCrossRefGoogle Scholar
- 20.James DG, Neville E, Siltzbach LE. A worldwide review of sarcoidosis. Ann N Y Acad Sci. 1976;278:321–34.PubMedCrossRefGoogle Scholar
- 21.Siltzbach LE, James DG, Neville E, Turiaf J, Battesti JP, Sharma OP, et al. Course and prognosis of sarcoidosis around the world. Am J Med. 1974;57(6):847–52.PubMedCrossRefGoogle Scholar
- 22.Sider L, Horton Jr ES. Hilar and mediastinal adenopathy in sarcoidosis as detected by computed tomography. J Thorac Imaging. 1990;5(2):77–80.PubMedCrossRefGoogle Scholar
- 23.Patil SN, Levin DL. Distribution of thoracic lymphadenopathy in sarcoidosis using computed tomography. J Thorac Imaging. 1999;14(2):114–7.PubMedCrossRefGoogle Scholar
- 24.Niimi H, Kang EY, Kwong JS, Carignan S, Muller NL. CT of chronic infiltrative lung disease: prevalence of mediastinal lymphadenopathy. J Comput Assist Tomogr. 1996;20(2):305–8.PubMedCrossRefGoogle Scholar
- 25.Gawne-Cain ML, Hansell DM. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis and tuberculosis: a CT study. Clin Radiol. 1996;51(4):263–7.PubMedCrossRefGoogle Scholar
- 26.Rabinowitz JG, Ulreich S, Soriano C. The usual unusual manifestations of sarcoidosis and the “hilar haze” – a new diagnostic aid. Am J Roentgenol Radium Ther Nucl Med. 1974;120(4):821–3.PubMedCrossRefGoogle Scholar
- 27.Romer FK. Presentation of sarcoidosis and outcome of pulmonary changes. Dan Med Bull. 1982;29(1):27–32.PubMedGoogle Scholar
- 28.Spann RW, Rosenow 3rd EC, DeRemee RA, Miller WE. Unilateral hilar or paratracheal adenopathy in sarcoidosis: a study of 38 cases. Thorax. 1971;26(3):296–9.PubMedCrossRefGoogle Scholar
- 29.Murdoch J, Muller NL. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol. 1992;159(3):473–7.PubMedCrossRefGoogle Scholar
- 30.McLoud TC, Putman CE, Pascual R. Eggshell calcification with systemic sarcoidosis. Chest. 1974;66(5):515–7.PubMedCrossRefGoogle Scholar
- 31.Webb WR. Thin-section CT, of the secondary pulmonary lobule: anatomy and the image – the 2004 Fleischner lecture. Radiology. 2006;239(2):322–38.PubMedCrossRefGoogle Scholar
- 32.McLoud TC, Epler GR, Gaensler EA, Burke GW, Carrington CB. A radiographic classification for sarcoidosis: physiologic correlation. Invest Radiol. 1982;17(2):129–38.PubMedCrossRefGoogle Scholar
- 33.Israel HL, Karlin P, Menduke H, DeLisser OG. Factors affecting outcome of sarcoidosis. Influence of race, extrathoracic involvement, and initial radiologic lung lesions. Ann N Y Acad Sci. 1986;465:609–18.PubMedCrossRefGoogle Scholar
- 34.Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic findings. Radiology. 1993;189(1):105–9.PubMedGoogle Scholar
- 35.Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT. Clin Radiol. 1996;51(11):807–10.PubMedCrossRefGoogle Scholar
- 36.Sharma OP, Hewlett R, Gordonson J. Nodular sarcoidosis: an unusual radiographic appearance. Chest. 1973;64(2):189–92.PubMedCrossRefGoogle Scholar
- 37.Battesti JP, Saumon G, Valeyre D, Amouroux J, Pechnick B, Sandron D, et al. Pulmonary sarcoidosis with an alveolar radiographic pattern. Thorax. 1982;37(6):448–52.PubMedCrossRefGoogle Scholar
- 38.McNicol MW, Luce PJ. Sarcoidosis in a racially mixed community. J R Coll Physicians Lond. 1985;19(3):179–83.PubMedGoogle Scholar
- 39.Malaisamy S, Dalal B, Bimenyuy C, Soubani AO. The clinical and radiologic features of nodular pulmonary sarcoidosis. Lung. 2009;187(1):9–15.PubMedCrossRefGoogle Scholar
- 40.Edelman RR, Johnson TS, Jhaveri HS, Kim D, Kasdon E, Frank HA, et al. Fatal hemoptysis resulting from erosion of a pulmonary artery in cavitary sarcoidosis. AJR Am J Roentgenol. 1985;145(1):37–8.PubMedCrossRefGoogle Scholar
- 41.Loh GA, Lettieri CJ, Shah AA. Bronchial arterial embolisation for massive haemoptysis in cavitary sarcoidosis. BMJ Case Rep. 2013. doi: 10.1136/bcr-2012-008268.Google Scholar
- 42.Leung AN, Brauner MW, Caillat-Vigneron N, Valeyre D, Grenier P. Sarcoidosis activity: correlation of HRCT findings with those of 67Ga scanning, bronchoalveolar lavage, and serum angiotensin-converting enzyme assay. J Comput Assist Tomogr. 1998;22(2):229–34.PubMedCrossRefGoogle Scholar
- 43.Brauner MW, Grenier P, Mompoint D, Lenoir S, de Cremoux H. Pulmonary sarcoidosis: evaluation with high-resolution CT. Radiology. 1989;172(2):467–71.PubMedGoogle Scholar
- 44.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.PubMedCrossRefGoogle Scholar
- 45.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.PubMedGoogle Scholar
- 46.Marchiori E, Zanetti G, Barreto MM, de Andrade FT, Rodrigues RS. Atypical distribution of small nodules on high resolution CT studies: patterns and differentials. Respir Med. 2011;105(9):1263–7.PubMedCrossRefGoogle Scholar
- 47.Voloudaki AE, Bouros DE, Froudarakis ME, Datseris GE, Apostolaki EG, Gourtsoyiannis NC. Crescentic and ring-shaped opacities. CT features in two cases of bronchiolitis obliterans organizing pneumonia (BOOP). Acta Radiol. 1996;37(6):889–92.PubMedCrossRefGoogle Scholar
- 48.Marchiori E, Zanetti G, Mano CM, Hochhegger B, Irion KL. The reversed halo sign: another atypical manifestation of sarcoidosis. Korean J Radiol. 2010;11(2):251–2.PubMedCrossRefGoogle Scholar
- 49.Zompatori M, Poletti V, Battista G, Diegoli M. Bronchiolitis obliterans with organizing pneumonia (BOOP), presenting as a ring-shaped opacity at HRCT (the atoll sign). A case report. Radiol Med. 1999;97(4):308–10.PubMedGoogle Scholar
- 50.Marten K, Rummeny EJ, Engelke C. The CT halo: a new sign in active pulmonary sarcoidosis. Br J Radiol. 2004;77(924):1042–5.PubMedCrossRefGoogle Scholar
- 51.Grenier P, Chevret S, Beigelman C, Brauner MW, Chastang C, Valeyre D. Chronic diffuse infiltrative lung disease: determination of the diagnostic value of clinical data, chest radiography, and CT and Bayesian analysis. Radiology. 1994;191(2):383–90.PubMedGoogle Scholar
- 52.Grenier P, Valeyre D, Cluzel P, Brauner MW, Lenoir S, Chastang C. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. Radiology. 1991;179(1):123–32.PubMedGoogle Scholar
- 53.Martin SG, Kronek LP, Valeyre D, Brauner N, Brillet PY, Nunes H, et al. High-resolution computed tomography to differentiate chronic diffuse interstitial lung diseases with predominant ground-glass pattern using logical analysis of data. Eur Radiol. 2010;20(6):1297–310.PubMedCrossRefGoogle Scholar
- 54.Moller DR. Pulmonary fibrosis of sarcoidosis. New approaches, old ideas. Am J Respir Cell Mol Biol. 2003;29(3 Suppl):S37–41.PubMedGoogle Scholar
- 55.Freundlich IM, Libshitz HI, Glassman LM, Israel HL. Sarcoidosis. Typical and atypical thoracic manifestations and complications. Clin Radiol. 1970;21(4):376–83.PubMedCrossRefGoogle Scholar
- 56.Primack SL, Hartman TE, Hansell DM, Muller NL. End-stage lung disease: CT findings in 61 patients. Radiology. 1993;189(3):681–6.PubMedGoogle Scholar
- 57.Davies CW, Tasker AD, Padley SP, Davies RJ, Gleeson FV. Air trapping in sarcoidosis on computed tomography: correlation with lung function. Clin Radiol. 2000;55(3):217–21.PubMedCrossRefGoogle Scholar
- 58.Hansell DM, Milne DG, Wilsher ML, Wells AU. Pulmonary sarcoidosis: morphologic associations of airflow obstruction at thin-section CT. Radiology. 1998;209(3):697–704.PubMedGoogle Scholar
- 59.Bartz RR, Stern EJ. Airways obstruction in patients with sarcoidosis: expiratory CT scan findings. J Thorac Imaging. 2000;15(4):285–9.PubMedCrossRefGoogle Scholar
- 60.Pena TA, Soubani AO, Samavati L. Aspergillus lung disease in patients with sarcoidosis: a case series and review of the literature. Lung. 2011;189(2):167–72.PubMedCrossRefGoogle Scholar
- 61.Pesle GD, Monod O. Bronchiectasis due to asperigilloma. Dis Chest. 1954;25(2):172–83.PubMedCrossRefGoogle Scholar
- 62.Chusid EL, Siltzbach LE. Sarcoidosis of the pleura. Ann Intern Med. 1974;81(2):190–4.PubMedCrossRefGoogle Scholar
- 63.Sharma OP, Gordonson J. Pleural effusion in sarcoidosis: a report of six cases. Thorax. 1975;30(1):95–101.PubMedCrossRefGoogle Scholar
- 64.Beekman JF, Zimmet SM, Chun BK, Miranda AA, Katz S. Spectrum of pleural involvement in sarcoidosis. Arch Intern Med. 1976;136(3):323–30.PubMedCrossRefGoogle Scholar
- 65.Wilen SB, Rabinowitz JG, Ulreich S, Lyons HA. Pleural involvement in sarcoidosis. Am J Med. 1974;57(2):200–9.PubMedCrossRefGoogle Scholar
- 66.Tommasini A, Di Vittorio G, Facchinetti F, Festi G, Schito V, Cipriani A. Pleural effusion in sarcoidosis: a case report. Sarcoidosis. 1994;11(2):138–40.PubMedGoogle Scholar
- 67.Soskel NT, Sharma OP. Pleural involvement in sarcoidosis. Curr Opin Pulm Med. 2000;6(5):455–68.PubMedCrossRefGoogle Scholar
- 68.Huggins JT, Doelken P, Sahn SA, King L, Judson MA. Pleural effusions in a series of 181 outpatients with sarcoidosis. Chest. 2006;129(6):1599–604.PubMedCrossRefGoogle Scholar
- 69.Littner MR, Schachter EN, Putman CE, Odero DO, Gee JB. The clinical assessment of roentgenographically atypical pulmonary sarcoidosis. Am J Med. 1977;62(3):361–8.PubMedCrossRefGoogle Scholar
- 70.Aberg H, Bah M, Waters AW. Sarcoidosis: complicated by chylothorax. Minn Med. 1966;49(7):1065–70.PubMedGoogle Scholar
- 71.Jarman PR, Whyte MK, Sabroe I, Hughes JM. Sarcoidosis presenting with chylothorax. Thorax. 1995;50(12):1324–5.PubMedCrossRefGoogle Scholar
- 72.Lengyel RJ, Shanley DJ. Recurrent chylothorax associated with sarcoidosis. Hawaii Med J. 1995;54(12):817–8.PubMedGoogle Scholar
- 73.Parker JM, Torrington KG, Phillips YY. Sarcoidosis complicated by chylothorax. South Med J. 1994;87(8):860–2.PubMedCrossRefGoogle Scholar
- 74.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.CrossRefGoogle Scholar
- 75.Froudarakis ME, Bouros D, Voloudaki A, Papiris S, Kottakis Y, Constantopoulos SH, et al. Pneumothorax as a first manifestation of sarcoidosis. Chest. 1997;112(1):278–80.PubMedCrossRefGoogle Scholar
- 76.Akelsson IG, Eklund A, Skold CM, Tornling G. Bilateral spontaneous pneumothorax and sarcoidosis. Sarcoidosis. 1990;7(2):136–8.PubMedGoogle Scholar
- 77.Popper HH, Klemen H, Colby TV, Churg A. Necrotizing sarcoid granulomatosis – is it different from nodular sarcoidosis? Pneumologie. 2003;57(5):268–71.PubMedCrossRefGoogle Scholar
- 78.Koss MN, Hochholzer L, Feigin DS, Garancis JC, Ward PA. Necrotizing sarcoid-like granulomatosis: clinical, pathologic, and immunopathologic findings. Hum Pathol. 1980;11(5 Suppl):510–9.PubMedGoogle Scholar
- 79.Rosen Y. Pathology of sarcoidosis. Semin Respir Crit Care Med. 2007;28(1):36–52.PubMedCrossRefGoogle Scholar
- 80.Liebow AA. The J. Burns Amberson lecture – pulmonary angiitis and granulomatosis. Am Rev Respir Dis. 1973;108(1):1–18.PubMedGoogle Scholar
- 81.Yeboah J, Afkhami M, Lee C, Sharma OP. Necrotizing sarcoid granulomatosis. Curr Opin Pulm Med. 2012;18(5):493–8.PubMedCrossRefGoogle Scholar
- 82.Handa T, Nagai S, Miki S, Fushimi Y, Ohta K, Mishima M, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006;129(5):1246–52.PubMedCrossRefGoogle Scholar
- 83.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.PubMedCrossRefGoogle Scholar
- 84.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.PubMedCrossRefGoogle Scholar
- 85.Nunes H, Uzunhan Y, Freynet O, Humbert M, Brillet PY, Kambouchner M, et al. Pulmonary hypertension complicating sarcoidosis. Presse Med. 2012;41(6 Pt 2):e303–16.PubMedCrossRefGoogle Scholar
- 86.Braun JJ, Kessler R, Constantinesco A, Imperiale A. 18F-FDG PET/CT in sarcoidosis management: review and report of 20 cases. Eur J Nucl Med Mol Imaging. 2008;35(8):1537–43.PubMedCrossRefGoogle Scholar
- 87.Keijsers RG, Verzijlbergen FJ, Oyen WJ, van den Bosch JM, Ruven HJ, van Velzen-Blad H, et al. 18F-FDG PET, genotype-corrected ACE and sIL-2R in newly diagnosed sarcoidosis. Eur J Nucl Med Mol Imaging. 2009;36(7):1131–7.PubMedCrossRefGoogle Scholar
- 88.Teirstein AS, Machac J, Almeida O, Lu P, Padilla ML, Iannuzzi MC. Results of 188 whole-body fluorodeoxyglucose positron emission tomography scans in 137 patients with sarcoidosis. Chest. 2007;132(6):1949–53.PubMedCrossRefGoogle Scholar
- 89.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.PubMedCrossRefGoogle Scholar
- 90.Keijsers RG, Verzijlbergen EJ, van den Bosch JM, Zanen P, van de Garde EM, Oyen WJ, et al. 18F-FDG PET as a predictor of pulmonary function in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2011;28(2):123–9.PubMedGoogle Scholar