Pulmonary Manifestations of Systemic Diseases

  • C. Schaefer-Prokop


The following review focuses on pulmonary manifestations of granulomatous diseases (sarcoidosis), connective tissue diseases, vasculitis (Wegener granulomatosis and Church-Strauss), and Goodpasture syndrome. Rare pulmonary manifestations associated with other diseases, such as tuberous sclerosis and lymphangioleiomyomatosis, Erdheim-Chester disease (rare non-Langerhans cell histiocytosis), storage diseases (Gaucher, Niemann Pick), systemic amyloidosis, and relapsing polychondritis, as well as manifestations of neoplastic diseases (e.g., lymphoma) are not considered herein. Langerhans cell histiocytosis shows a pulmonary involvement that is associated with a systemic manifestation only in children and not in adults.


Idiopathic Pulmonary Fibrosis High Resolution Compute Tomography Usual Interstitial Pneumonia Progressive Systemic Sclerosis Cryptogenic Organize Pneumonia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Suggested Reading

Connective-Tissue Disorders

  1. Akira M, Hara H, Sakatani M (1999) Interstitial lung disease in association with polymyositis/dermatomyositis: long term follow up CT evaluation in seven patients. Radiology 210:333–338PubMedGoogle Scholar
  2. Hansell DM, Armstrong P, Lynch DA, Page McAdams H (2005) Imaging diseases of the chest, 4th edn. Mosby, London, p 534Google Scholar
  3. Aquino SL, Webb RW, Golden J (1994) Bronchiolitis obliterans associated with rheumatoid arthritis: findings on HRCT and dynamic expiratory CT. J Comput Assist Tomogr 18:555–558PubMedCrossRefGoogle Scholar
  4. Bankier AA, Kiener HP, Wiesmayr MN et al (1995) Discrete lung involvement in systemic lupus erythematosus: CT assessment. Radiology 196:835–840PubMedGoogle Scholar
  5. Demosthenos B, Wells AU, Nicholson Ag et al (2002) Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med 165:1581–1586CrossRefGoogle Scholar
  6. Desai SR, Nicholson AG, Stewart S et al (1997) Benign pulmonary lymphocytic infiltration and amyloidosis: computed tomographic and pathologic features in three cases. J Thorac Imag 12:215–220Google Scholar
  7. Desai SR, Veeraraghavan S, Hansell DM et al (2004) CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia. Radiology 232:560–567PubMedCrossRefGoogle Scholar
  8. Douglas WW, Tazelaar HD, Hartman TE et al (2001) Polymyositis-Dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 164:1182–1185PubMedGoogle Scholar
  9. Franquet T, Gimenez A, Monill JM et al (1997) Primary Sjögren’s syndrome and associated lung disease. AJR Am J Roentgenol 169:655–658PubMedGoogle Scholar
  10. Holzmann H, Jacobi V, Werbner RJ, Stahl E (1994) Lungenmanifestation bei progressiver systemischer Sklerodermie: CT Befunde. Hautarzt 45:471–475PubMedCrossRefGoogle Scholar
  11. Honda O, Jokhoh T, Ichikado K et al (1999) Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high resolution CT. AJR Am J Roentgenol 173:71–74PubMedGoogle Scholar
  12. Hunninghake GW, Fauci AS (1979) Pulmonary involvement in the collagen vascular diseases. Am Rev Respir Dis 119:471–503PubMedGoogle Scholar
  13. Ikezoe J, Johkoh T, Nohno N et al (1996) High resolution CT findings of lung disease in patients with polymyositis/dermatomyositis. J Thorac Imag 11:250–259CrossRefGoogle Scholar
  14. Johkoh T, Ikezoe J, Kohno N (1994) High resolution CT and pulmonary function tests in collagen vascular disease: comparison with idiopathic pulmonary fibrosis. Eur J Radiol 18:113–121PubMedCrossRefGoogle Scholar
  15. Johkoh T, Müller NL, Pickford HA et al (1999) Lymphocytic interstitial pneumonia: thin section CT findings in 22 patients. Radiology 212:567–572PubMedGoogle Scholar
  16. Kocheril SV, Appleton E, Somers EC et al (2005) Comparison of disease progression and mortality of connective tissue diseaserelated interstitial lung disease and idiopathic interstitial pneumonia. Arthritis and Rheumatism 53:549PubMedCrossRefGoogle Scholar
  17. Lakhanapal S, Lie JT, Conn DL et al (1987) Pulmonary disease in polymyositis / dermatomyosistis: a clinicopathological analysis of 65 cases. Ann Rheum Dis 47:23–9Google Scholar
  18. Lynch JP, Hunninghake GW (1992) Pulmonary complications of collagen vascular disease. Ann Rev Med 43:17–35PubMedCrossRefGoogle Scholar
  19. Mayberry JP, Primack SL, Müller NL (2000) Thoracic manifestations of systemic autoimmune diseases: radiographic and high resolution CT findings. Radiographics 20:1623–1635PubMedGoogle Scholar
  20. Meyer CA, Pina JS, Taillon D et a (1997) Inspiratory and expiratory high resolution CT findings in a patient with Sjögren’s syndrome and cystic lung disease. AJR Am J Roentgenol 168:101–103PubMedGoogle Scholar
  21. Nicholson AG, Colby TV, Wells AU (2002) Histopathological approach to patterns of interstitial pneumonia in patients with connective tissue disorders. Sarcoidosis Vasc Diff Lung Dis 19:10–17Google Scholar
  22. Ooi GC, Ngan H, Peh WC et al (1997) Systemic lupus erythematosus patients with respiratory symptoms: the value of HRCT. Clin Radiol 52:775–781PubMedCrossRefGoogle Scholar
  23. Owens GR, Fino GJ, Hernert DL (1983) Pulmonary function in progressive systemic sclerosis: comparison of CREST syndrome variant with diffuse scleroderma. Chest 84:546–550PubMedGoogle Scholar
  24. Parambil JG, Myers JL, Ryu JH (2006) Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connetive tissue diseases. Chest 130:553PubMedCrossRefGoogle Scholar
  25. Remy-Jardin M, Remy J, Cortet B et al (1994) Lung changes in rheumatoid arthritis: CT findings. Radiology 193:375–382PubMedGoogle Scholar
  26. Remy-Jardin M, Remy J, Wallaert B et al (1993) Pulmonary involvement in progressive systemic sclerosis: sequential evaluation with CT, pulmonary function tests, and bronchoalveolar lavage. Radiology 188:499–506PubMedGoogle Scholar
  27. Reuter M, Schnabel A, Wesner F et al (1998) Pulmonary Wegener’s granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest 114:500–506PubMedGoogle Scholar
  28. Scheja A, Akesson A, Wollmer P et al (1993) Early pulmonary disease in systemic sclerosis: a comparison between carbon monoxide transfer and static lung compliance. Ann Rheum Dis 52:725PubMedCrossRefGoogle Scholar
  29. Schurawitzki H, Stiglbaur R, Graninger W et al (1990) Interstitial lung disease in progressive systemic sclerosis: high resolution CT versus radiography. Radiology 176:755–759PubMedGoogle Scholar
  30. Sperber M (ed) (1999) Diffuse lung disorder. Springer, London, Berlin, Heidelberg, p 329Google Scholar
  31. Tanaka N, Kim JS, Newell JD et al (2004) Rheumatoid arthritis-related lung diseases: CT findings. Radiology 232:81PubMedCrossRefGoogle Scholar
  32. Tansey D, Wells AU, Colby TV et al (2004) variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 44:585PubMedCrossRefGoogle Scholar
  33. Tazelaar HD, Viggiano RW, Pickersgill J et al (1990) Interstitial lung diseases in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 141:727–733PubMedGoogle Scholar
  34. Wells AU Cullinan P, Hansell DM et al (1994) Fibrosing alveolitis associated with systematic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 149:1583–1589PubMedGoogle Scholar
  35. Wells AU, duBois RM (1993) Bronchiolitis in association with connective tissue disorders. Clin Chest Med 14:655–666PubMedGoogle Scholar
  36. Wells AU, Hansell DM, Corrin B et al (1992) High resolution computed tomography as a predictor of lung histology in systematic sclerosis. Thorax 47:738–742PubMedGoogle Scholar
  37. Wells AU, Rubens MB, duBois RM, Hansell DM (1993a). Serial CT in fibrosing alveolitis: prognostic significance of the initial pattern. AJR Am J Roentgenol 161:1159–65PubMedGoogle Scholar
  38. Wells AU, Hansell DM, Rubens MB et al (1993b) The predictive value of appearances on thin section computed tomography in fibrosing alveolitis. Am Rev Respir Dis 148:1076–1082PubMedGoogle Scholar
  39. Wells AU, Hansell DM, Rubens MB et al (1997) Functional impairment in long cryptogenic fibrosing alveolitis and fibrosing alveolitis. Am Rev Resp Dis 148:1076–1082Google Scholar


  1. Attali P, Begum R, Romdhane HB et al (1998) Pulmonary Wegener’s granulomatosis: changes at follow up CT. Eur Radiol 8:1009–1013PubMedCrossRefGoogle Scholar
  2. Cordier JF, Valeyre D, Guillevin L et al (1990) Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 97:906–912PubMedGoogle Scholar
  3. Fenlon HM, Doran M, Sant SM et al (1996) High resolution CT in systemic lupus erythematosus. AJR Am J Roentgenol 166:301PubMedGoogle Scholar
  4. Haworth SJ, Savage CO, Carr D et al (1985) Pulmonary haemorrhage complicating Wegener’s granulomatosis and microscopic polyarteritis. Br Med J (Clin Res Ed) 290:1175–1178CrossRefGoogle Scholar
  5. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: Proposal of an international conference. Arthris Rheumatol 37:187CrossRefGoogle Scholar
  6. Jennette JC, Falk RJ (2007) Nosology of primary vasculitis. Curr Opin Rheumatol 19:10–16PubMedCrossRefGoogle Scholar
  7. Marten K, Schnyder P, Schirg E et al (2005) Pattern-based differential diagnosis in pulmonary vasculitis using volumetric CT. AJR Am J Roentgenol 184:720–733PubMedGoogle Scholar
  8. Specks U, Deremee RA (1990) Granulomatous vasculitis. Wegener’s granulomatosis and Churg Strauss syndrome. Rheum Dis Clin North Am 16:377–397PubMedGoogle Scholar
  9. Susanto I, Peters JI (1997) Acute lupus pneumonitis with normal chest radiograph. Chest 111:1781–1783PubMedGoogle Scholar
  10. Worthy SA, Muller NL, Hansell DM, Flower CD (1998) Churg strauss syndrome: the spectrum of pulmonary CT findings in 17 Patients. AJR Am J Roentgenol 170:297–300PubMedGoogle Scholar

Copyright information

© Springer-Verlag Italia 2007

Authors and Affiliations

  • C. Schaefer-Prokop
    • 1
  1. 1.Department of RadiologyAcademic Medical CenterAmsterdamThe Netherlands

Personalised recommendations