• Robina K. CokerEmail author


Sarcoidosis is a multisystem inflammatory disease that commonly affects the lungs, but its precise cause remains unknown. Although mortality is rare, sarcoidosis can cause significant morbidity, including debilitating fatigue and systemic upset, limiting the ability to work or carry out everyday activities. Mild cases may resolve spontaneously and never require treatment, but vital organ involvement is an indication for immunosuppression. The optimal choice of agent and duration of treatment remain unclear. Since sarcoidosis frequently presents to respiratory specialists, they need to be aware of the potential for extra-pulmonary disease and how to recognise it. Diagnosis and management of extra-pulmonary disease requires close collaboration between specialists in different disciplines.


Sarcoidosis Interstitial lung disease Granuloma Erythema nodosum Corticosteroids Immunosuppression Methotrexate 


  1. 1.
    Rybicki BA, Iannuzzi MC, Frederick MM, Thompson BW, Rossman MD, Bresnitz EA, et al. Familial aggregation of sarcoidosis: a case-control etiologic study of sarcoidosis (ACCESS). Am J Respir Crit Care Med. 2001;164(11):2085–91.CrossRefPubMedGoogle Scholar
  2. 2.
    Scadding JG. Prognosis of intrathoracic sarcoidosis in England. Br Med J. 1961;2:1165–72.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Birnie DH, Sauer WH, Bogun F, Cooper JM, Culver DA, Duvernoy CS, et al. HRS expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis. Heart Rhythm. 2014;11(7):1305–23.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Lower EE, Harman S, Baughman RP. A randomized, double-blind placebo controlled trial of dexmethylphenidate hydrochloride (d-MPH) for sarcoidosis associated fatigue. Chest. 2008;133:1189–95.CrossRefPubMedGoogle Scholar
  5. 5.
    Lower EE, Malhotra A, Surdulescu V, Baughman RP. Armodafinil for sarcoidosis-associated fatigue: a double-blind, placebo-controlled, crossover trial. J Pain Symptom Manag. 2013;45(2):159–69.CrossRefGoogle Scholar
  6. 6.
    Wells AU, Hirani N. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand, and the Irish Thoracic Society. Thorax. 2008;63(Suppl V):v1–58.CrossRefPubMedGoogle Scholar
  7. 7.
    Gibson GJ, Prescott RJ, Muers MF, Middleton WG, Mitchell DN, Connolly CK, et al. British Thoracic Society Sarcoidosis study: effects of long-term corticosteroid treatment. Thorax. 1996;51(3):238–47.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Cremers JP, Drent M, Bast A, Shigemitsu H, Baughman RP, Valeyre D, et al. Multinational evidence-based World Association of Sarcoidosis and Other Granulomatous Disorders recommendations for the use of methotrexate in sarcoidosis: integrating systematic literature research and expert opinion of sarcoidologists worldwide. Curr Opin Pulm Med. 2013;19:545–61.CrossRefPubMedGoogle Scholar
  9. 9.
    Müller-Quernheim J, Kienast K, Held M, Pfeifer S, Costabel U. Treatment of chronic sarcoidosis with an azathioprine/prednisolone regimen. Eur Respir J. 1999;14:1117.CrossRefPubMedGoogle Scholar
  10. 10.
    British Tuberculosis Association. Chloroquine in the treatment of sarcoidosis. A report from the Research Committee of the British Tuberculosis Association. Tubercle. 1967;48(4):257–72.CrossRefGoogle Scholar
  11. 11.
    Baltzan M, Mehta S, Kirkham TH, Cosio MG. Randomized trial of prolonged chloroquine therapy in advanced pulmonary sarcoidosis. Am J Respir Crit Care Med. 1999;160:192–7.CrossRefPubMedGoogle Scholar
  12. 12.
    Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest. 2005;127:1064–71.CrossRefPubMedGoogle Scholar
  13. 13.
    Baughman RP, Drent M, Kavuru M, Judson MA, Costabel U, du Bois RM, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006;174:795–802.CrossRefPubMedGoogle Scholar
  14. 14.
    Grossman JM, Gordon R, Ranganath VK, Deal C, Caplan L, Chen W, et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken). 2010;62(11):1515–26.CrossRefGoogle Scholar
  15. 15.
    Bone and Tooth Society, National Osteoporosis Society, Royal College of Physicians. Glucocorticoid-induced osteoporosis: guidelines for prevention and treatment. London: RCP; 2002.Google Scholar
  16. 16.
    National Institute for Health and Care Excellence. Osteoporosis: assessing the risk of fragility fracture. NICE clinical guideline 146. 2012. Available at:
  17. 17.
    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

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Respiratory MedicineHammersmith Hospital, Imperial College Healthcare NHS TrustLondonUK

Personalised recommendations