Sarcoidosis: Epidemiology, Etiology, Pathogenesis, and Diagnosis

Authors

  • Fachri Setiawan Universitas Indonesia, Jawa Barat, Indonesia
  • Fariz Nurwidya Universitas Indonesia, Jawa Barat, Indonesia

DOI:

https://doi.org/10.58344/jws.v3i2.544

Keywords:

noncaseating granuloma, interstitial lung disease, sarcoidosis, Lofgren's syndrome

Abstract

Interstitial lung disease is a lung condition with wide variations. There are over 200 disorders classified under interstitial lung disease, including sarcoidosis. Sarcoidosis is a systemic disease characterized by the formation of non-caseating granulomas involving the lungs, lymphatic system, skin, and eyes. Clinical manifestations of sarcoidosis vary greatly, ranging from asymptomatic to severe symptoms with uncertain prognosis. The cause of sarcoidosis remains unknown, though several hypotheses suggest its association with genetic, environmental, infectious, and autoimmune factors. The main pathogenesis involves the formation of non-caseating granulomas involving various types of innate and adaptive immune cells. Based on its clinical presentation, sarcoidosis is classified into two types: Lofgren syndrome and non-Lofgren syndrome. Diagnosis of this disease can be established through clinical examination, radiological imaging, and biopsy revealing non-caseating granulomas. Corticosteroid therapy remains the primary treatment option, alongside immunosuppressive and biological therapies for advanced-stage sarcoidosis.

References

Arkema, E. V., & Cozier, Y. C. (2020). Sarcoidosis epidemiology: Recent estimates of incidence, prevalence and risk factors. Current Opinion in Pulmonary Medicine, 26(5), 527–534. https://doi.org/10.1097/MCP.0000000000000715

Bernardinello, N., Petrarulo, S., Balestro, E., Cocconcelli, E., Veltkamp, M., & Spagnolo, P. (2021). Pulmonary sarcoidosis: Diagnosis and differential diagnosis. Diagnostics, 11(9), 1–14. https://doi.org/10.3390/diagnostics11091558

Cottin, V., Hirani, N. A., Hotchkin, D. L., Nambiar, A. M., Ogura, T., Otaola, M., Skowasch, D., Park, J. S., Poonyagariyagorn, H. K., & Wuyts, W. (2018). Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. European Respiratory Review, 27(150).

Cozzi, D., Bargagli, E., Calabrò, A. G., Torricelli, E., Giannelli, F., Cavigli, E., & Miele, V. (2018). Atypical HRCT manifestations of pulmonary sarcoidosis. Radiologia Medica, 123(3), 174–184. https://doi.org/10.1007/s11547-017-0830-y

Crouser, E. D., Maier, L. A., Baughman, R. P., Abston, E., Bernstein, R. C., Blankstein, R., Bonham, C. A., Chen, E. S., Culver, D. A., Drake, W., Drent, M., Gerke, A. K., Ghobrial, M., Govender, P., Hamzeh, N., James, W. E., Judson, M. A., Kellermeyer, L., Knight, S., … Wilson, K. C. (2020). Diagnosis and Detection of Sarcoidosis An Official American Thoracic Society Clinical Practice Guideline. American Journal of Respiratory and Critical Care Medicine, 201(8), 26–51. https://doi.org/10.1164/RCCM.202002-0251ST

Grunewald, J., Grutters, J. C., Arkema, E. V., Saketkoo, L. A., Moller, D. R., & Müller-Quernheim, J. (2019). Sarcoidosis. Nature Reviews Disease Primers, 5(1), 1–22. https://doi.org/10.1038/s41572-019-0096-x

Jain, R., Yadav, D., Puranik, N., Guleria, R., & Jin, J. O. (2020). Sarcoidosis: Causes, diagnosis, clinical features, and treatments. Journal of Clinical Medicine, 9(4), 1–21. https://doi.org/10.3390/jcm9041081

Lee, S., Birnie, D., & Dwivedi, G. (2020). Current perspectives on the immunopathogenesis of sarcoidosis. Respiratory Medicine, 173(August), 106–161. https://doi.org/10.1016/j.rmed.2020.106161

Newman, K. L., & Newman, L. S. (2012). Occupational causes of sarcoidosis. Current Opinion in Allergy and Clinical Immunology, 12(2), 145–150. https://doi.org/10.1097/ACI.0b013e3283515173

Polverino, F., Balestro, E., & Spagnolo, P. (2020). Clinical presentations, pathogenesis, and therapy of sarcoidosis: State of the art. Journal of Clinical Medicine, 9(8), 1–17. https://doi.org/10.3390/jcm9082363

Putra, A. C., Andarini, S. L., Susanto, A. D., Damayanti, T., Samoedro, E., Priyonugroho, G., Septiyanti, D., Fajriwan, & Bernida, I. (2017). Penyakit paru interstisial.pdf (A. C. Putra & S. L. Andarini (eds.)). Perhimpuan Dokter Paru Indonesia.

Sakthivel, P., & Bruder, D. (2017). Mechanism of granuloma formation in sarcoidosis. Current Opinion in Hematology, 24(1), 59–65. https://doi.org/10.1097/MOH.0000000000000301

Sève, P., Pacheco, Y., Durupt, F., Jamilloux, Y., Gerfaud-Valentin, M., Isaac, S., Boussel, L., Calender, A., Androdias, G., Valeyre, D., & El Jammal, T. (2021). Sarcoidosis: A clinical overview from symptoms to diagnosis. Cells, 10(4), 1–33. https://doi.org/10.3390/cells10040766

Sikjær, M. G., Hilberg, O., Ibsen, R., & Løkke, A. (2021). Sarcoidosis: A nationwide registry-based study of incidence, prevalence and diagnostic work-up. Respiratory Medicine, 187(July), 1–8. https://doi.org/10.1016/j.rmed.2021.106548

Spagnolo, P. (2015). Sarcoidosis: a Critical Review of History and Milestones. Clinical Reviews in Allergy and Immunology, 49(1), 15–19. https://doi.org/10.1007/s12016-015-8480-0

Spagnolo, P., Rossi, G., Trisolini, R., Sverzellati, N., Baughman, R. P., & Wells, A. U. (2018). Pulmonary sarcoidosis. The Lancet Respiratory Medicine, 6(5), 389–402. https://doi.org/10.1016/S2213-2600(18)30064-X

Tana, C., Donatiello, I., Caputo, A., Tana, M., Naccarelli, T., Mantini, C., Ricci, F., Ticinesi, A., Meschi, T., Cipollone, F., & Giamberardino, M. A. (2022). Clinical features, histopathology and differential diagnosis of sarcoidosis. Cells, 11(1), 1–16. https://doi.org/10.3390/cells11010059

Zissel, G., & Müller-Quernheim, J. (2016). Specific antigen(s) in sarcoidosis: A link to autoimmunity? European Respiratory Journal, 47(3), 707–709. https://doi.org/10.1183/13993003.01791-2015

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Published

2024-02-29

How to Cite

Setiawan, F., & Nurwidya, F. . (2024). Sarcoidosis: Epidemiology, Etiology, Pathogenesis, and Diagnosis. Journal of World Science, 3(2), 166–182. https://doi.org/10.58344/jws.v3i2.544