Skip to main content
SpringerLink
Log in

Pulmonary Manifestations of Systemic Diseases

  • Chapter
Diseases of the Heart and Chest, Including Breast 2011–2014

  • 965 Accesses

Abstract

Lung involvement in systemic disease may be a manifestation of the underlying pathological process, a complication of the underlying disease or related to treatment. Lung pathology is dominant in certain diseases, such as sarcoidosis or Wegener’s granulomatosis, but may be only rarely present, for example, in Henoch-Schönlein purpura. Lung involvement has a profound effect on prognosis and may be challenging to accurately diagnose. Imaging often plays a central part when lung involvement is suspected clinically. In the following, we focus on the more common entities that frequently develop pulmonary abnormalities, such as sarcoidosis, connective tissue diseases, vasculitis and inflammatory bowel disease. Rare pulmonary manifestations of tuberous sclerosis and lymphangioleiomyomatosis, Erdheim-Chester disease, storage diseases (Gaucher, Niemann Pick) or systemic amyloidosis as well as manifestations within neoplastic diseases (e.g. lymphoma) are not considered in this chapter. Langerhans cell histiocytosis shows pulmonary involvement associated with a systemic manifestation only in children but not in adults.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight 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

Suggested Reading

Sarcoidosis

  • Brauner MW, Lenoir S, Grenier P et al (1992) Pulmonary sarcoidosis: CT assessment of lesion reversibility. Radiology 182:349–354

    PubMed  CAS  Google Scholar 

  • 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 

  • Handa T, Nagai S, Fushimi Y et al (2006) Clinical and radiographic indices associated with airflow limitations in patients with sarcoidosis. Chest 130(6):1851–1856

    Article  PubMed  Google Scholar 

  • Nakatsu M, Hatabu H, Morikawa K et al (2002) Large coalescent nodules in pulmonary sarcoidosis: “sarcoid galaxy sign”. AJR Am J Roentgenol 178(6): 1389–1393

    Article  PubMed  Google Scholar 

Collagen Vascular Diseases

  • American Thoracic Society (2002) American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 165:277–304

    Article  Google Scholar 

  • Hwang JH, Misumi S, Sahin H et al (2009) Computed tomographic features of idiopathic fibrosing interstitial pneumonia: comparison with pulmonary fibrosis related to collagen vascular disease. J Comput Assist Tomogr 33:410–415.

    Article  PubMed  Google Scholar 

  • Kim EA, Lee KS, Johkoh T et al (2002) Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. RadioGraphics 22:S151–S165

    Article  PubMed  Google Scholar 

  • Lynch DA (2009) Lung disease related to collagen vascular diseases. J Thorac Imag 24:299–309

    Article  Google Scholar 

Rheumatoid Arthritis

  • Aquino SL, Webb WR, Golden J (1994) Bronchiolitis obliterans associated with rheumatoid arthritis: findings on HRCT and dynamic expiratory CT. J Comput Assist Tomog 18:555–558

    Article  CAS  Google Scholar 

  • Arakawa H, Honma K, Shida H et al (2003) Computed tomography findings of Caplan syndrome. J Comput Assist Tomogr 27:758–760

    Article  PubMed  Google Scholar 

  • Bouros D, Hatzakis K, Labrakis H, Zeibecoglou K (2002) Association of malignancy with diseases causing interstitial pulmonary changes. Chest 121:1278–1289

    Article  PubMed  Google Scholar 

  • Gabbay E, Tarala R, Will R et al (1997) Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med 156:528–535

    Article  PubMed  CAS  Google Scholar 

  • Golden MR, Katz RS, Balk RA, Golden HE (1995) The relationship of preexisting lung disease to the development of methotrexate pneumonitis in patients with rheumatoid arthritis. J Rheumatol 22:1043–1047

    PubMed  CAS  Google Scholar 

  • Hilliquin P, Renoux M, Perrot S et al (1996) Occurrence of pulmonary complications during methotrexate therapy in rheumatoid arthritis. Br J Rheumatol 35:441–445

    Article  PubMed  CAS  Google Scholar 

  • Metafratzi ZM, Georgiadis AN, Ioannidou CV et al (2007) Pulmonary involvement in patients with early rheumatoid arthritis. Scand J Rheumatol 36:338–344

    Article  PubMed  CAS  Google Scholar 

  • Mori S, Cho I, Koga Y, Sugimoto M (2008) Comparison of pulmonary abnormalities on high-resolution computed tomography in patients with early versus longstanding rheumatoid arthritis. J Rheumatol 35:1513–1521

    PubMed  Google Scholar 

  • Mutlu GM, Mutlu EA, Bellmeyer A, Rubinstein I (2006) Pulmonary adverse events of anti-tumor necrosis factor-alpha antibody therapy. Am J Med 119:639–646

    Article  PubMed  CAS  Google Scholar 

  • Remy-Jardin M, Remy J, Cortet B et al (1994) Lung changes in rheumatoid arthritis: CT findings. Radiology 193:375–382

    PubMed  CAS  Google Scholar 

  • Perez T, Remy-Jardin M, Cortet B (1998) Airways involvement in rheumatoid arthritis: clinical, functional, and HRCT findings. Am J Respir Crit Care Med 157:1658–1665

    Article  PubMed  CAS  Google Scholar 

  • Tanaka N, Kim JS, Newell JD et al (2004) Rheumatoid arthritis-related lung diseases: CT findings. Radiology 232:81–91

    Article  PubMed  Google Scholar 

Systemic Sclerosis

  • Bouros D, 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 165:1581–1586

    Article  PubMed  Google Scholar 

  • Fischer A, Misumi S, Curran-Everett D et al (2007) Pericardial abnormalities predict the presence of echocardiographically defined pulmonary arterial hypertension in systemic sclerosis-related interstitial lung disease. Chest 131:988–992

    Article  PubMed  Google Scholar 

  • Goldin JG, Lynch DA, Strollo DC et al (2008) High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 134:358–367

    Article  PubMed  Google Scholar 

  • Kim DS, Yoo B, Lee JS et al (2002) The major histopathologic pattern of pulmonary fibrosis in scleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis 19:121–127

    PubMed  Google Scholar 

  • Pearson JE, Silman AJ (2003) Risk of cancer in patients with scleroderma. Ann Rheum Dis 62:697–699

    Article  PubMed  CAS  Google Scholar 

  • 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 188:499–506

    PubMed  CAS  Google Scholar 

  • Wells AU, Cullinan P, Hansell DM et al (1994) Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Care Med 149:1583–1590

    Article  PubMed  CAS  Google Scholar 

Lupus Erythematosus

  • Bankier AA, Kiener HP, Wiesmayr MN et al (1995) Discrete lung involvement in systemic lupus erythematosus: CT assessment. Radiology 196:835–840

    PubMed  CAS  Google Scholar 

  • Fenlon HM, Doran M, Sant SM, Breatnach E (1996) High-resolution chest CT in systemic lupus erythematosus. AJR Am J Roentgenol 166:301–307

    Article  PubMed  CAS  Google Scholar 

  • Swigris JJ, Fischer A, Gillis J (2008) Pulmonary and thrombotic manifestations of systemic lupus erythematosus. Chest 133:271–280

    Article  PubMed  Google Scholar 

  • Wiedemann HP, Matthay RA (1992) Pulmonary manifestations of systemic lupus erythematosus. J Thorac Imaging 7:1–18

    Article  PubMed  CAS  Google Scholar 

Dermatomyositis/Polymyositis

  • Arakawa H, Yamada H, Kurihara Y et al (2003) Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation. Chest 123:1096–1103

    Article  PubMed  Google Scholar 

  • Fischer A, Swigris JJ, du Bois RM et al (2009) Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med 103:1719–1724

    Article  PubMed  Google Scholar 

  • Mino M, Noma S, Taguchi Y et al (1997) Pulmonary involvement in polymyositis and dermatomyositis: sequential evaluation with CT. AJR Am J Roentgenol 169:83–87

    Article  PubMed  CAS  Google Scholar 

Sjögren Syndrome

  • Franquet T, Gimenez A, Monill JM et al (1997) Primary Sjogren’s syndrome and associated lung disease: CT findings in 50 patients. AJR Am J Roentgenol 169:655–658

    Article  PubMed  CAS  Google Scholar 

  • Honda O, Johkoh T, Ichikado K et al (1999) Differential diagnosis of lymphocytic interstitial pneumonia and malignant lymphoma on high-resolution CT. AJR Am J Roentgenol 173:71–74

    Article  PubMed  CAS  Google Scholar 

  • Jeong YJ, Lee KS, Chung MP et al (2004) Amyloidosis and lymphoproliferative disease in Sjogren syndrome: thin-section computed tomography findings and histopathologic comparisons. J Comput Assist Tomogr 28:776–781

    Article  PubMed  Google Scholar 

  • Johkoh T, Müller NL, Pickford HA et al (1999) Lymphocytic interstitial pneumonia: thin-section CT findings in 22 patients. Radiology 212:567–572

    PubMed  CAS  Google Scholar 

  • Taouli B, Brauner MW, Mourey I et al (2002) Thin-section chest CT findings of primary Sjogren’s syndrome: correlation with pulmonary function. Eur Radiol 12:1504–1511

    Article  PubMed  Google Scholar 

Mixed Connective Tissue Disease

  • Bodolay E, Szekanecz Z, Devenyi K et al (2005) Evaluation of interstitial lung disease in mixed connective tissue disease (MCTD). Rheumatology (Oxford) 44:656–661

    Article  CAS  Google Scholar 

  • Fagan KA, Badesch DB (2002) Pulmonary hypertension associated with connective tissue disease. Prog Cardiovasc Dis 45:225–234

    Article  PubMed  Google Scholar 

  • Kozuka T, Johkoh T, Honda O et al (2001) Pulmonary involvement in mixed connective tissue disease: high-resolution CT findings in 41 patients. J Thorac Imaging 16:94–98

    Article  PubMed  CAS  Google Scholar 

Ankylosing Spondylitis

  • Hillerdal G (1983) Ankylosing spondylitis lung disease — an underdiagnosed entity? Eur J Respir Dis 64:437–441

    PubMed  CAS  Google Scholar 

  • Pamuk ON, Harmandar O, Tosun B et al (2005) A patient with ankylosing spondylitis who presented with chronic necrotising aspergillosis: report on one case and review of the literature. Clin Rheumatol 24:415–419

    Article  PubMed  Google Scholar 

Vasculitis

  • Attali P, Begum R, Romdhane HB et al (1998) Pulmonary Wegener’s granulomatosis: changes at follow up CT. Eur Radiol 8:1009–1113

    Article  PubMed  CAS  Google Scholar 

  • Chung MP, Yi CA, Lee HY et al (2010) Imaging of pulmonary vasculitis. Radiology 255:322–341

    Article  PubMed  Google Scholar 

  • Cordier JF, Valeyre D, Guillevin L et al (1990) Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 97:906–912

    Article  PubMed  CAS  Google Scholar 

  • Haworth SJ, Savage COS, Carr D et al (1985) Pulmonary hemorrhage complicating Wegener’s granulomatosis and microscopic polyarteritis. BMJ 290:1175–1178

    Article  Google Scholar 

  • Jennette JC, Falk RJ (2007) Nosology of primary vasculitis. Curr Opin Rheumatol 19:10–16

    Article  PubMed  Google Scholar 

  • Marten K, Schnyder P, Schirg E et al (2005) Pattern-based differential diagnosis in pulmonary vasculitis using volumetric CT. AJR Am J Roentgenol 184:720–733

    Article  PubMed  Google Scholar 

  • Specks U, Deremee RA (1990) Granulomatous vasculitis. Wegener’s granulomatosis and Churg Strauss syndrome. Rheum Dis Clin North Am 16:377–397

    PubMed  CAS  Google Scholar 

  • Susanto I, Peters JI (1997) Acute lupus pneumonitis with normal chest radiograph. Chest 111:1781–1783

    Article  PubMed  CAS  Google Scholar 

  • 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 170:297–300

    Article  PubMed  CAS  Google Scholar 

Inflammatory Bowel Disease

  • Camus P, Piard F, Ashcroft T et al (1993) The lung in inflammatory bowel disease. Medicine (Baltimore) 72:151–183

    CAS  Google Scholar 

  • Garg K, Lynch DA, Newell JD (1993) Inflammatory airways disease in ulcerative colitis: CT and high-resolution CT features. J Thorac Imaging 8:159–163

    Article  PubMed  CAS  Google Scholar 

  • Kelly MG, Frizelle FA, Thornley PT et al (2006) Inflammatory bowel disease and the lung: is there a link between surgery and bronchiectasis? Int J Colorectal Dis 21:754–757

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, National Jewish Health, Denver, CO, USA

    David A. Lynch

  2. Department of Radiology, Meander Medical Center, Amersfoort, Netherlands

    Cornelia Schaefer-Prokop

Authors
  1. David A. Lynch
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cornelia Schaefer-Prokop
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Radiology, University Hospital, Zurich, Switzerland

    J. Hodler

  2. Nuclear Medicine, University Hospital, Zurich, Switzerland

    G. K. von Schulthess

  3. Kilchberg/Zurich, Switzerland

    Ch. L. Zollikofer

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer-Verlag Italia

About this chapter

Cite this chapter

Lynch, D.A., Schaefer-Prokop, C. (2011). Pulmonary Manifestations of Systemic Diseases. In: Hodler, J., von Schulthess, G.K., Zollikofer, C.L. (eds) Diseases of the Heart and Chest, Including Breast 2011–2014. Springer, Milano. https://doi.org/10.1007/978-88-470-1938-6_18

Download citation

  • DOI: https://doi.org/10.1007/978-88-470-1938-6_18

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-1937-9

  • Online ISBN: 978-88-470-1938-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation