Volume 31, Issue 1 (May 2024)                   JBUMS 2024, 31(1): 1-14 | Back to browse issues page

Research code: not applicable
Ethics code: not applicable


XML Persian Abstract Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Ebrahimzadeh F, Samadi S, Mirfeizi Z, Hashemzadeh K. A clinical overview of sarcoidosis: Pathogenesis, symptoms, diagnosis and treatment. JBUMS 2024; 31 (1) :1-14
URL: http://journal.bums.ac.ir/article-1-3338-en.html
1- Department of Internal Medicine, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
2- Rheumatic Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
3- Rheumatic Diseases Research Center, Mashhad University of Medical Sciences, Mashhad, Iran , Hashemzadehk@mums.ac.ir
Abstract:   (334 Views)
Sarcoidosis is a granulomatous disorder characterized by non-caseating granulomas in multiple organs and an unknown cause. This chronic inflammatory disease is heterogeneous in terms of manifestations, duration, and severity. Although its aetiology is not well understood, there are indications of the genetic basis and the involvement of specific microorganisms and vitamin D in the development of this disease. Sarcoidosis can affect individuals of any age; however, it is more commonly observed in young adults and middle-aged subjects. The clinical course of the disease is unpredictable; nevertheless, in general, it can manifest as pulmonary, cardiac, cutaneous, ocular, skeletal-muscular, neurological, and renal involvement. Pulmonary involvement occurs in most patients and respiratory failure is the most common cause of death associated with sarcoidosis. Although numerous biomarkers have been evaluated in recent decades for patients with sarcoidosis, the diagnostic method is complex and no single diagnostic test can confirm the disease. Future research should focus on the combination of biomarkers and more refined imaging techniques, such as computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET). By utilizing these techniques and markers, it may be possible to design diagnostic and therapeutic approaches. Corticosteroids are considered the mainstay of treatment for most patients. Moreover, novel therapies, including the administration of rituximab and repository corticotropin injection (a combination of adrenocorticotropic hormone analogs and other pituitary peptides [RCI, Acthar® Gel]), have been reported to be effective in some cases.
Full-Text [PDF 631 kb]   (190 Downloads)    
Type of Study: Review | Subject: Rheumatology
Received: 2023/10/8 | Accepted: 2024/04/28 | ePublished ahead of print: 2024/05/27 | ePublished: 2024/06/4

References
1. Bittencourt MD, Anjos AB, Brito FC, Unger DA. Cutaneous sarcoidosis with livedo-like manifestation. An Bras Dermatol. 2019; 94(4): 493-4. DOI: 10.1590/abd1806-4841.20198173 [DOI:10.1590/abd1806-4841.20198173] [PMID] []
2. Seve P, Pacheco Y, Durupt F, Jamilloux Y, Gerfaud-ValentinM I. Sarcoidosis: a clinical overview from symptoms to diagnosis. Cells.2021; 10 (4): 766. DOI: 10.3390/cells10040766 [DOI:10.3390/cells10040766] [PMID] []
3. Facco M, Cabrelle A, Teramo A, Olivieri V, Gnoato M, Teolato S, et al. Sarcoidosis is a Th1/Th17 multisystem disorder. Thorax. 2011; 66(2): 144-50. DOI: 10.1136/thx.2010.140319 [DOI:10.1136/thx.2010.140319] [PMID]
4. Grunewald J, Spagnolo P, Wahlström J, Eklund AJCria, immunology. Immunogenetics of disease-causing inflammation in sarcoidosis. Clin Rev Allergy Immunol. 2015; 49(1): 19-35. DOI: 10.1007/s12016-015-8477-8 [DOI:10.1007/s12016-015-8477-8] [PMID]
5. Palmucci S, Torrisi SE, Caltabiano DC, Puglisi S, Lentini V, Grassedonio E, et al. Clinical and radiological features of extra-pulmonary sarcoidosis: a pictorial essay. Insights Imaging. 2016; 7(4): 571-87. DOI: 10.1007/s13244-016-0495-4 [DOI:10.1007/s13244-016-0495-4] [PMID] []
6. Kempf W, Zollinger T, Sachs M, Ullmer E, Cathomas G, Dirnhofer S, et al. Granulomas are a source of interleukin-33 expression in pulmonary and extrapulmonary sarcoidosis. Hum Pathol. 2014; 45(11): 2202-10. DOI: 10.1016/j.humpath.2014.07.011 [DOI:10.1016/j.humpath.2014.07.011] [PMID]
7. Bechman K, Christidis D, Walsh S, Birring SS, Galloway J. A review of the musculoskeletal manifestations of sarcoidosis. Rheumatology (Oxford). 2018; 57(5): 777-83. DOI: 10.1093/rheumatology/kex317. PMID: 28968840. [DOI:10.1093/rheumatology/kex317] [PMID]
8. Bargagli E, Prasse AJI, medicine e. Sarcoidosis: a review for the internist. Intern Emerg Med. 2018; 13(3): 325-31. DOI: 10.1007/s11739-017-1778-6 [DOI:10.1007/s11739-017-1778-6]
9. Seyed AAF, Siamak G, Azita TN, Aydin PK, Ehsan KNL, Alireza J, et al. Comparison of serum interleukin-10 level of fungal exposure among patients with pulmonary sarcoidosis and healthy people. Sarcoidosis Vasc Diffuse Lung Dis. 2018; 35(4): 294-8. DOI: 10.36141/svdld.v35i4.6757.
10. Xiong Y, Kullberg S, Garman L, Pezant N, Ellinghaus D, Vasila V, et al. Sex differences in the genetics of sarcoidosis across European and African ancestry populations. Front Med (Lausanne). 2023; 10: 1132799. DOI: 10.3389/fmed.2023.1132799 [DOI:10.3389/fmed.2023.1132799] [PMID] []
11. Jayakrishnan B, Al-Busaidi N, Al-Mubaihsi S, Al-Rawas OAJAotm. Sarcoidosis in the Middle East. Ann Thorac Med. 2019; 14(2): 106-15. DOI: 10.4103/atm.ATM_227_18 [DOI:10.4103/atm.ATM_227_18] [PMID] []
12. Spagnolo P, Rossi G, Trisolini R, Sverzellati N, Baughman RP, Wells AU. Pulmonary sarcoidosis. Lancet Respir Med. 2018; 6(5): 389-402. DOI: 10.1016/S2213-2600(18)30064-X [DOI:10.1016/S2213-2600(18)30064-X] [PMID]
13. Ungprasert P, Ryu JH, Matteson EL. Clinical manifestations, diagnosis, and treatment of sarcoidosis. Mayo Clin Proc Innov Qual Outcomes. 2019; 3(3): 358-75. DOI: 10.1016/j.mayocpiqo.2019.04.006 [DOI:10.1016/j.mayocpiqo.2019.04.006] [PMID] []
14. El Jammal T, Jamilloux Y, Gerfaud-Valentin M, Valeyre D, Sève PJT, management cr. Refractory sarcoidosis: a review. Ther Clin Risk Manag. 2020; 16: 323-45. DOI: 10.2147/TCRM.S192922 [DOI:10.2147/TCRM.S192922] [PMID] []
15. Mirfeizi Z, Hashemzadeh S, Hashemzadeh K. Undiagnosed acrodermatitis continua leading to amputations of the distal phalanxes. Int J Rheum Dis. 2008; 11: 308-10. DOI: 10.1111/j.1756-185X.2008.00381.x [DOI:10.1111/j.1756-185X.2008.00381.x]
16. Rezazadeh M, Jokar MH, Mehrnaz Aghili S, Mirfeizi Z, Mahmoudi M, Morovatdar N, Hashemzadeh K. Association between levels of serum and urinary B cell-activating factor and systemic lupus erythematosus disease activity. Arch Rheumatol. 2023; 38(3): 429-40. DOI: 10.46497/ArchRheumatol.2023.9549. PMID: 38046245; PMCID: PMC10689013. [DOI:10.46497/ArchRheumatol.2023.9549] [PMID] []
17. Hena KM. Sarcoidosis epidemiology: race matters. Front Immunol. 2020; 11: 537382. DOI: 10.3389/fimmu.2020.537382 [DOI:10.3389/fimmu.2020.537382] [PMID] []
18. Krishna Y, Christou L, Khzouz J, Hussain R, Heimann H, Coupland SE. Optic nerve head sarcoidosis mimicking an intraocular tumour, and occurring as the first manifestation of neuro-ocular sarcoidosis. Am J Ophthalmol Case Rep. 2020; 20: 100988. DOI: 10.1016/j.ajoc.2020.100988. [DOI:10.1016/j.ajoc.2020.100988] [PMID] []
19. Rossides M, Darlington P, Kullberg S, Arkema EV. Sarcoidosis: Epidemiology and clinical insights. J Intern Med. 2023; 293(6): 668-80. DOI: 10.1111/joim.13629 [DOI:10.1111/joim.13629] [PMID]
20. Ramachandraiah V, Aronow W, Chandy D. Pulmonary sarcoidosis: an update. Postgrad Med. 2017; 129(1): 149-58. DOI: 10.1080/00325481.2017.1251818 [DOI:10.1080/00325481.2017.1251818] [PMID]
21. Mirfeizi Z, Abadi RN. Hepatosplenic Sarcoidosis with Pulmonary Involvement: A Case Report on a Concomitant Extrapulmonary Site. Journal of Case Reports in Practice. 2022; 9(1).
22. Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013; 10(4): 362-70. DOI: 10.1513/AnnalsATS.201303-069FR [DOI:10.1513/AnnalsATS.201303-069FR] [PMID]
23. Teirstein AT, Morgenthau AS. "End‐stage" Pulmonary Fibrosis in Sarcoidosis. Mt Sinai J Med. 2009; 76(1): 30-6. DOI: 10.1002/msj.20090 [DOI:10.1002/msj.20090] [PMID]
24. Gupta R, Judson MA, Baughman RP. Management of Advanced Pulmonary Sarcoidosis. Am J Respir Crit Care Med. 2022; 205(5): 495-506. DOI: 10.1164/rccm.202106-1366CI [DOI:10.1164/rccm.202106-1366CI] [PMID]
25. Birnie DH, Nery PB, Ha AC, Beanlands RS. Cardiac Sarcoidosis. J Am Coll Cardiol. 2016; 68(4): 411-21. DOI: 10.1016/j.jacc.2016.03.605 [DOI:10.1016/j.jacc.2016.03.605] [PMID]
26. Ricci F, Mantini C, Grigoratos C, Bianco F, Bucciarelli V, Tana C, et al. The Multi-modality Cardiac Imaging Approach to Cardiac Sarcoidosis. Curr Med Imaging Rev. 2019; 15(1): 10-20. DOI: 10.2174/1573405614666180522074320 [DOI:10.2174/1573405614666180522074320] [PMID]
27. Yatsynovich Y, Dittoe N, Petrov M, Maroz N. Cardiac sarcoidosis: a review of contemporary challenges in diagnosis and treatment. Am J Med Sci. 2018; 355(2): 113-25. DOI: 10.1016/j.amjms.2017.08.009 [DOI:10.1016/j.amjms.2017.08.009] [PMID]
28. Zhou Y LE, Li H, Farhey Y, Baughman RP. Clinical characteristics of patients with bone sarcoidosis. Semin Arthritis Rheum. 2017; 47(1): 143-8.DOI: 10.1016/j.semarthrit.2017.02.004. [DOI:10.1016/j.semarthrit.2017.02.004] [PMID]
29. Matsou A, Tsaousis KT. Management of chronic ocular sarcoidosis: challenges and solutions. Clin Ophthalmol. 2018; 12: 519-32. DOI: 10.2147/OPTH.S128949 [DOI:10.2147/OPTH.S128949] [PMID] []
30. Yang SJ, Salek S, Rosenbaum JT. Ocular sarcoidosis: new diagnostic modalities and treatment. Curr Opin Pulm Med. 2017; 23(5): 458-67. DOI: 10.1097/MCP.0000000000000409. PMID: 28622198; PMCID: PMC5642043. [DOI:10.1097/MCP.0000000000000409] [PMID] []
31. Stehlé T, Boffa JJ, Lang P, Desvaux D, Sahali D, Audard V. Atteintes rénales de la sarcoïdose [Kidney involvement in sarcoidosis]. Rev Med Interne. 2013; 34(9): 538-44. DOI: 10.1016/j.revmed.2012.10.009. [DOI:10.1016/j.revmed.2012.10.009] [PMID]
32. Bergner R, Löffler C.. Renal sarcoidosis: approach to diagnosis and management. Curr Opin Pulm Med. 2018; 24(5): 513-20. DOI: 10.1097/MCP.0000000000000504 [DOI:10.1097/MCP.0000000000000504] [PMID]
33. Tejera Segura B HS, Mateo L, Pego-Reigosa JM, Carnicero Iglesias M, Olivé A. Síndrome de Löfgren: estudio de 80 casos [Löfgren syndrome: a study of 80 cases]. Med Clin (Barc). 2014 Aug 19; 143(4):166-9. Spanish. DOI: 10.1016/j.medcli.2014.02.029. Epub 2014 May 22. PMID: 24855899. Löfgren syndrome: a study of 80 cases. 2014; 143(4): 166-9. [DOI:10.1016/j.medcli.2014.02.029] [PMID]
34. Kirresh O, Schofield J, George G. Acute sarcoidosis: Löfgren's syndrome. Br J Hosp Med (Lond). 2015;76(3): 154-8. DOI: 10.12968/hmed.2015.76.3.154. PMID: 25761805. [DOI:10.12968/hmed.2015.76.3.154] [PMID]
35. Holgado S, Mateo L, Pego-Reigosa JM, Olivé A. Löfgren syndrome: a study of 80 cases. Med Clin (Barc). 2014; 143(4): 166-9. DOI: 10.1016/j.medcli.2014.02.029 [DOI:10.1016/j.medcli.2014.02.029] [PMID]
36. Noe MH, Rosenbach M. Cutaneous sarcoidosis. Curr Opin Pulm Med. 2017; 23(5): 482-6. DOI: 10.1097/MCP.0000000000000402 [DOI:10.1097/MCP.0000000000000402] [PMID]
37. Karadağ AS, Parish LC. Sarcoidosis: A great imitator. Clin Dermatol. 2019; 37(3): 240-54. DOI: 10.1016/j.clindermatol.2019.01.005 [DOI:10.1016/j.clindermatol.2019.01.005] [PMID]
38. Ruocco E, Gambardella A, Langella GG, Lo Schiavo A, Ruocco V. Cutaneous sarcoidosis: an intriguing model of immune dysregulation. Int J Dermatol. 2015; 54(1): 1-12. DOI: 10.1111/ijd.12566. Epub 2014 Oct 14. PMID: 25312788. [DOI:10.1111/ijd.12566] [PMID]
39. Saghafi M, Rezaieyazdi Z, Nabavi Sh, Mirfeizi Z, Sahebari M, Salari M. HTLV-1 seroprevalance in sarcoidosis. A clinical and laboratory study in northeast of Iran. Int J Rheum Dis. 2018; 21(6): 1309-13. DOI: 10.1111/1756-185X.13009 [DOI:10.1111/1756-185X.13009] [PMID]
40. Shen J, Lackey E, Shah S. Neurosarcoidosis: Diagnostic Challenges and Mimics A Review. Curr Allergy Asthma Rep. 2023; 23(7): 399-410. DOI: 10.1007/s11882-023-01092-z [DOI:10.1007/s11882-023-01092-z] [PMID] []
41. Chen ES, Moller DR. Etiologies of sarcoidosis. 2015; 49(1): 6-18. DOI: 10.1007/s12016-015-8481-z [DOI:10.1007/s12016-015-8481-z] [PMID]
42. Mirfeizi Z, Sahebari M, Nabavi S, Salari M, Saghafi M, Rezaieyazdi Z, et al. Human leukocyte antigen-DRB1 alleles in patients with sarcoidosis from Northeast Iran. Rheumatology Research. 2018; 3(3): 119-22. DOI: 10.22631/rr.2018.69997.1052 [DOI:10.22631/rr.2018.69997.1052]
43. Kempisty A, Lewandowska A, Kuś J. Disturbances of calcium metabolism and vitamin D supplementation in sarcoidosis - two-way street. Pol Merkur Lekarski. 2018; 44(261): 147-9. [Polish]. PMID: 29601566.
44. Kamphuis LS, Bonte‐Mineur F, van Laar JA, van Hagen PM, van Daele PL. Calcium and vitamin D in sarcoidosis: is supplementation safe? J Bone Miner Res. 2014; 29(11): 2498-503. DOI: 10.1002/jbmr.2262. PMID: 24753153. [DOI:10.1002/jbmr.2262] [PMID]
45. Polverino F BE, Spagnolo P. Clinical Presentations, Pathogenesis, and Therapy of Sarcoidosis: State of the Art. J Clin Med. 2020; 9(8): 2363. DOI: 10.3390/jcm9082363. PMID: 32722050; PMCID: PMC7465477 [DOI:10.3390/jcm9082363] [PMID] []
46. Hashemzadeh K, Fatemipour M, Mirfeizi SZ, Jokar M, Shariati Sarabi Z, Hatef Fard MR, et al. Serum B cell activating factor (BAFF) and sarcoidosis activity. Arch Rheumatol. 2021; 36(1): 72-9. DOI: 10.46497/ArchRheumatol.2021.8013
47. Ramos-Casals M Retamozo S, Sisó-Almirall A, Pérez-Alvarez R, Pallarés L, Brito-Zerón P. Clinically-useful serum biomarkers for diagnosis and prognosis of sarcoidosis. Expert Rev Clin Immunol. 2019; 15(4): 391-405. DOI: 10.1080/1744666X.2019.1568240. Epub 2019 Jan 26. PMID: 30632406.15(4): 391-405. [DOI:10.1080/1744666X.2019.1568240] [PMID]
48. Kraaijvanger R, Janssen Bonás M, Vorselaars ADM, Veltkamp M. Biomarkers in the Diagnosis and Prognosis of Sarcoidosis: Current Use and Future Prospects. Front Immunol. 2020; 11: 1443.DOI: 10.3389/fimmu.2020.01443. PMID: 32760396; PMCID: PMC7372102.11:1443. [DOI:10.3389/fimmu.2020.01443] [PMID] []
49. Keijsers RG, Verzijlbergen FJ, Oyen WJ, van den Bosch JM, Ruven HJ, van Velzen-Blad H, et al. 18 F-FDG PET, genotype-corrected ACE and sIL-2R in newly diagnosed sarcoidosis.Eur J Nucl Med Mol Imaging. 2009; 36(7): 1131-7. DOI: 10.1007/s00259-009-1097-x [DOI:10.1007/s00259-009-1097-x] [PMID]
50. Vorselaars AD, Verwoerd A, van Moorsel CH, Keijsers RG, Rijkers GT, Grutters JC. Prediction of relapse after discontinuation of infliximab therapy in severe sarcoidosis. Eur Respir J. 2014; 43(2): 602-9. DOI: 10.1183/09031936.00055213 [DOI:10.1183/09031936.00055213] [PMID]
51. Baughman RP, Valeyre D, Korsten P, Mathioudakis AG, Wuyts WA, Wells A, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J. 2021; 58(6): 2004079. DOI: 10.1183/13993003.04079-2020 [DOI:10.1183/13993003.04079-2020] [PMID]
52. Fraser E, Weeratunga P, Ho L-P. Management of Sarcoidosis: When to Treat, How to Treat and for How Long? Curr Pulmonol Rep. 2022; 11(4): 151-9. DOI: 10.1007/s13665-022-00298-2 [DOI:10.1007/s13665-022-00298-2]
53. Kashyap S, Bhardwaj M. New sarcoidosis guidelines: Are we near to perfection? Lung India. 2022; 39(3): 217-219. DOI: 10.4103/lungindia.lungindia_159_22 [DOI:10.4103/lungindia.lungindia_159_22] [PMID] []
54. Rahaghi FF, Baughman RP, Saketkoo LA, Sweiss NJ, Barney JB, Birring SS, et al. Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur Respir Rev. 2020; 29(155): 190146. DOI: 10.1183/16000617.0146-2019 [DOI:10.1183/16000617.0146-2019] [PMID] []
55. Baughman RP, Wells A. Advanced sarcoidosis. Current opinion in pulmonary medicine. 2019; 25(5): 497-504. [DOI:10.1097/MCP.0000000000000612] [PMID]
56. Baughman RP, Barriuso R, Beyer K, Boyd J, Hochreiter J, Knoet C, et al. Sarcoidosis: patient treatment priorities. ERJ Open Res. 2018; 4(4): 00141-2018. DOI: 10.1183/23120541.00141-2018 [DOI:10.1183/23120541.00141-2018] [PMID] []
57. Papanikolaou IC, Antonakis E, Pandi A. State-of-the-art treatments for sarcoidosis. Methodist Debakey Cardiovasc J. 2022; 18(2): 94-105. DOI: 10.14797/mdcvj.1068 [DOI:10.14797/mdcvj.1068] [PMID] []
58. Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O. Early treatment of stage II sarcoidosis improves 5-year pulmonary function. Chest. 2002; 121(1): 24-31. DOI: 10.1378/chest.121.1.24 [DOI:10.1378/chest.121.1.24] [PMID]
59. Broos CE, Poell LH, Looman CW, CCM J, Grootenboers MJ, Heller R, et al. No evidence found for an association between prednisone dose and FVC change in newly-treated pulmonary sarcoidosis. Respir Med. 2018; 138: S31-S7. DOI: 10.1016/j.rmed.2017.10.022 [DOI:10.1016/j.rmed.2017.10.022] [PMID]
60. Judson MA. Advances in the diagnosis and treatment of sarcoidosis. F1000prime rep. 2014;6: 89. DOI: 10.12703/P6-89 [DOI:10.12703/P6-89] [PMID] []
61. James WE BRTosgteERCPJ-dE. Treatment of sarcoidosis: grading the evidence. Expert Rev Clin Pharmacol. 2018; 11(7): 677-87. DOI: 10.1080/17512433.2018.1486706 [DOI:10.1080/17512433.2018.1486706] [PMID]
62. West SG. Current management of sarcoidosis I: pulmonary c, and neurologic manifestations. Curr Opin Rheumatol. 2018; 30(3): 243-8. DOI: 10.1097/BOR.0000000000000489. PMID: 29389828. [DOI:10.1097/BOR.0000000000000489] [PMID]

Add your comments about this article : Your username or Email:
CAPTCHA

Send email to the article author


Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

© 2024 CC BY-NC 4.0 | Journal of Birjand University of Medical Sciences

Designed & Developed by : Yektaweb