Skip to main content
SpringerLink
Log in

Sarcoidosis

  • Chapter
Primer on the Rheumatic Diseases

  • 104k Accesses

Abstract

Sarcoidosis is a systemic inflammatory disorder characterized by the presence of noncaseating granulomatous inflammation in affected organs (1). The etiology of sarcoidosis remains undetermined, the clinical manifestations of this disease are protean, and a diagnosis of sarcoidosis is often made by the exclusion of other processes. What helps distinguish sarcoidosis from other systemic disorders is a consideration of clinical presentation and natural history, confirmatory biopsy, and appropriate response to therapy. Although this disease most commonly affects the lungs, virtually any part of the body may be affected, and the presence and behavior of characteristic extrapulmonary manifestations may assist in supporting a diagnosis of sarcoidosis.

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 99.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 129.99
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

References

  1. Hunninghake GW, Costabel U, Ando M, et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vas Diffuse Lung Dis 1999;16:149–173.

    CAS  Google Scholar 

  2. Kitaichi M. Prevalence of sarcoidosis around the world. Sarcoidosis Vas Diffuse Lung Dis 1998;15:16–18.

    CAS  Google Scholar 

  3. Siltzbach LE, James DG, Neville E, et al. Course and prognosis of sarcoidosis around the world. Am J Med 1974;57:847–852.

    Article  CAS  PubMed  Google Scholar 

  4. Nelson RS. Sarcoidosis in the armed forces. Am J Med Sci 1953;226:131–138.

    Article  CAS  PubMed  Google Scholar 

  5. Pietinalho A, Hiraga Y, Hosoda Y, Lofroos AB, Yamaguchi M, Selroos O. The frequency of sarcoidosis in Finland and Hokkaido, Japan. A comparative epidemiological study. Sarcoidosis 1995;12:61–67.

    CAS  Google Scholar 

  6. Hagerstrand I, Linell F. The prevalence of sarcoidosis in the autopsy material from a Swedish town. Acta Med Scand 1964;425:171–174.

    CAS  Google Scholar 

  7. Reid JD. Sarcoidosis in coroner’s autopsies: a critical evaluation of diagnosis and prevalence from Cuyahoga County, Ohio. Sarcoidosis Vas Diffuse Lung Dis 1998;15:44–51.

    CAS  Google Scholar 

  8. Rybicki BA, Major M, Popovich J Jr, Maliarik MJ, Iannuzzi MC. Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization. Am J Epidemiol 1997;145:234–241.

    CAS  PubMed  Google Scholar 

  9. Schurmann M, Reichel P, Muller-Myhsok B, Schlaak M, Muller-Quernheim J, Schwinger E. Results from a genome-wide search for predisposing genes in sarcoidosis. Am J Respir Crit Care Med 2001;164:840–846.

    CAS  PubMed  Google Scholar 

  10. Valentonyte R, Hampe J, Huse K, et al. Sarcoidosis is associated with a truncating splice site mutation in BTNL2. Nat Genet 2005;37:357–364.

    Article  CAS  PubMed  Google Scholar 

  11. Rybicki BA, Hirst K, Iyengar SK, et al. A sarcoidosis genetic linkage consortium: the sarcoidosis genetic analysis (SAGA) study. Sarcoidosis Vas Diffuse Lung Dis 2005;22:115–122.

    Google Scholar 

  12. Rybicki BA, Walewski JL, Maliarik MJ, Kian H, Iannuzzi MC. The BTNL2 gene and sarcoidosis susceptibility in African Americans and Whites. Am J Hum Genet 2005;77:491–499.

    Article  CAS  PubMed  Google Scholar 

  13. Moller DR. Rare manifestations of sarcoidosis. In: Drent M, Costabel U, eds. Sarcoidosis. Vol 10. Wakefield, UK: European Respiratory Society Journals, Ltd.; 2005:233–250.

    Google Scholar 

  14. Gran JT, Bohmer E. Acute sarcoid arthritis: a favourable outcome? A retrospective survey of 49 patients with review of the literature. Scand J Rheumatol 1996;25:70–73.

    Article  CAS  PubMed  Google Scholar 

  15. Kaplan H. Sarcoid arthritis. A review. Arch Intern Med 1963;112:924–935.

    CAS  Google Scholar 

  16. Wilcox A, Bharadwaj P, Sharma OP. Bone sarcoidosis. Curr Opin Rheumatol 2000;12:321–330.

    Article  CAS  PubMed  Google Scholar 

  17. Kennedy PT, Zakaria N, Modawi SB, et al. Natural history of hepatic sarcoidosis and its response to treatment. Eur J Gastroenterol Hepatol 2006;18:721–726.

    Article  CAS  PubMed  Google Scholar 

  18. Lower EE, Smith JT, Martelo OJ, Baughman RP. The anemia of sarcoidosis. Sarcoidosis 1988;5:51–55.

    CAS  PubMed  Google Scholar 

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

    CAS  Google Scholar 

  20. Ardehali H, Howard DL, Hariri A, et al. A positive endomyocardial biopsy result for sarcoid is associated with poor prognosis in patients with initially unexplained cardiomyopathy. Am Heart J 2005;150:459–463.

    Article  PubMed  Google Scholar 

  21. Voorter CE, Drent M, Hoitsma E, Faber KG, van den Berg-Loonen EM. Association of HLA DQB1 0602 in sarcoidosis patients with small fiber neuropathy. Sarcoidosis Vasc Diffuse Lung Dis 2005;22:129–132.

    PubMed  Google Scholar 

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

    CAS  Google Scholar 

  23. Sharma OP. Vitamin D, calcium, and sarcoidosis. Chest 1996;109:535–539.

    Article  CAS  PubMed  Google Scholar 

  24. Chen ES, Moller DR. Cytokines and chemokines in sarcoidosis. In: Baughman R, ed. Sarcoidosis. Vol 210. New York: Taylor & Francis Group; 2006:123–161.

    Google Scholar 

  25. Forman JD, Klein JT, Silver RF, Liu MC, Greenlee BM, Moller DR. Selective activation and accumulation of oligoclonal V beta-specific T cells in active pulmonary sarcoidosis. J Clin Invest 1994;94:1533–1542.

    Article  CAS  PubMed  Google Scholar 

  26. Grunewald J, Janson CH, Eklund A, et al. Restricted V alpha 2.3 gene usage by CD4+ T lymphocytes in bronchoalveolar lavage fluid from sarcoidosis patients correlates with HLA-DR3. Eur J Immunol 1992;22:129–135.

    Article  CAS  PubMed  Google Scholar 

  27. Song Z, Marzilli L, Greenlee BM, et al. Mycobacterial catalase-peroxidase is a tissue antigen and target of the adaptive immune response in systemic sarcoidosis. J Exp Med 2005;201:755–767.

    Article  CAS  PubMed  Google Scholar 

  28. Ebe Y, Ikushima S, Yamaguchi T, et al. Proliferative response of peripheral blood mononuclear cells and levels of antibody to recombinant protein from Propionibacterium acnes DNA expression library in Japanese patients with sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2000;17:256–265.

    CAS  PubMed  Google Scholar 

  29. Newman LS, Rose CS, Bresnitz EA, et al. A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med 2004;170:1324–1330.

    Article  PubMed  Google Scholar 

  30. Judson MA, Baughman RP, Thompson BW, et al. Two year prognosis of sarcoidosis: the ACCESS experience. Sarcoidosis Vas Diffuse Lung Dis 2003;20:204–211.

    Google Scholar 

  31. Baughman RP, Drent M, Kavuru M, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med 2006;174:795–802.

    Article  CAS  PubMed  Google Scholar 

  32. Utz JP, Limper AH, Kalra S, et al. Etanercept for the treatment of stage II and III progressive pulmonary sarcoidosis. Chest 2003;124:177–185.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine, Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Edward S. Chen MD (Assistant Professor)

Authors
  1. Edward S. Chen MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Arthritis Foundation, Atlanta, GA, USA

    John H. Klippel MD (President and CEO) (President and CEO)

  2. Massachusetts General Hospital, Boston, MA, USA

    John H. Stone MD, MPH (Associate Physician, Deputy Editor for Rheumatology) (Associate Physician, Deputy Editor for Rheumatology)

  3. Division of Rheumatology & Women’s Health, University of Kentucky, Lexington, KY, USA

    Leslie J. Crofford MD (Gloria W. Singletary Professor of Internal Medicine, Chief) (Gloria W. Singletary Professor of Internal Medicine, Chief)

  4. Arthritis Foundation, Atlanta, GA, USA

    Patience H. White MD, MA (Chief Public Health Officer) (Chief Public Health Officer)

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Chen, E.S. (2008). Sarcoidosis. In: Klippel, J.H., Stone, J.H., Crofford, L.J., White, P.H. (eds) Primer on the Rheumatic Diseases. Springer, New York, NY. https://doi.org/10.1007/978-0-387-68566-3_27

Download citation

  • DOI: https://doi.org/10.1007/978-0-387-68566-3_27

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-35664-8

  • Online ISBN: 978-0-387-68566-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation