Sarcoidosis: federal clinical guidelines for diagnosis and treatment

Sarcoidosis is a multi-organ granulomatosis  of unknown  origin. Modern  diagnostic methods  allow detecting  this disease at an early stage. The absence of specific markers requires a comprehensive  approach  to diagnosis based on comparison  of radiation,  clinical, morphological  and functional data. The course of sarcoidosis without damage to the respiratory system presents significant difficulties. It is extremely important  to understand the time and means of starting Sarcoidosis’s treatment to avoid the early initiation  of hormones  and cytostatics and, on the other hand,  to timely respond to progression and threatening  conditions.  Methods. Clinical recommendations are based on the analysis of Russian and English publications of the latest sarcoidosis research. The target audience of these clinical guidelines are therapists, general practitioners, pulmonologists, TB doctors, rheumatologists,  dermatologists,  radiation diagnosticians,  immunologists,  and clinical pharmacologists.  Each thesis-recommendation for diagnosis and treatment is evaluated on an 1 to 5 scale of levels of evidence and an A, B, C scale of the grades of recommendations. The clinical guidelines also contain comments and explanations for the theses-recommendations, diagnostic algorithms, treatment strategies, reference materials on the use of recommended drugs. Conclusion. Current information  on epidemiology, clinical manifestations,  diagnosis and management  strategies for patients with sarcoidosis are covered in the presented clinical guidelines. Approved by the decision of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (2022).

1. Vizel’ A.A, ed. [Sarcoidosis]. Moscow: Atmosphera; 2010 (in Russian).

2. ATS/ERS/WASOG statement on sarcoidosis. Sarcoidosis statement committee. American Thoracic Society. European Respiratory Society. World Association for Sarcoidosis and Other Granulomatous Disorders. Eur. Respir. J. 1999; 14 (4): 735–737. DOI: 10.1034/j.1399-3003.1999.14d02.x.

3. Landi C., Carleo A., Cillis G., Rottoli P. Sarcoidosis: proteomics and new perspectives for improving personalized medicine. Expert Rev. Proteomics. 2018; 15 (10): 829–835. DOI: 10.1080/14789450.2018.1528148.

4. Besnard V., Calender A., Bouvry D. et al. G908R NOD2 variant in a family with sarcoidosis. Respir. Res. 2018; 19 (1): 44. DOI: 10.1186/s12931-018-0748-5.

5. Khomenko A.G., Shvayger O., eds. [Sarcoidosis]. Moscow: Meditsina; 1982 (in Russian).

6. Zhao M.M., Du S.S., Li Q.H. et al. High throughput 16SrRNA gene sequencing reveals the correlation between Propionibacterium acnes and sarcoidosis. Respir. Res. 2017; 18 (1): 28. DOI: 10.1186/s12931-017-0515-z.

7. van Dee L., Stehouwer M., van Bemmel T. Systemic sarcoidosis associated with exposure to Borrelia burgdorferi in a 21-year-old man. Eur. J. Case Rep. Intern. Med. 2018; 5 (10): 000942. DOI: 10.12890/2018_000942.

8. Esteves T., Aparicio G., Garcia-Patos V. Is there any association between sarcoidosis and infectious agents? a systematic review and meta-analysis. BMC Pulm. Med. 2016; 16 (1): 165. DOI: 10.1186/s12890-016-0332.

9. García Ródenas M.D.M., Gayá García-Manso I., García Sevila R. Sarcoidosis associated with Interferon beta treatment. Med. Clin. (Barc). 2019; 153 (5): e21–22. DOI: 10.1016/j.medcli.2018.11.021.

10. Vizel’ A.A., Vizel’ I.Yu. [Sarcoidosis and interferons: pathogenesis and iatrogeny]. Prakticheskaya pul’monologiya. 2017; (1): 46–50. Available at: https://atmosphere-ph.ru/modules/Magazines/articles/pulmo/pp_1_2017_46.pdf (in Russian).

11. Bargagli E., Prasse A. Sarcoidosis: a review for the internist. Intern. Emerg. Med. 2018; 13 (3): 325–331. DOI: 10.1007/s11739-017-1778-6.

12. Baughman R.P., Culver D.A., Judson M.A. A concise review of pulmonary sarcoidosis. Am. J. Respir. Crit. Care Med. 2011; 183 (5): 573–581. DOI: 10.1164/rccm.201006-0865ci.

13. Patterson K.C., Chen E.S. The pathogenesis of pulmonary sarcoidosis and implications for treatment. Chest. 2018; 153 (6): 1432–1442. DOI: 10.1016/j.chest.2017.11.030.

14. Hu Y., Yibrehu B., Zabini D., Kuebler W.M. Animal models of sarcoidosis. Cell Tissue Res. 2017; 367 (3): 651–661. DOI: 10.1007/s00441-016-2526-3.

15. Dubaniewicz A. [“Danger theory”as a common mechanism of sarcoidosis induction by infectious and non-infectious factors – a role of environmental factors and autoimmunity]. Pol. Merkur. Lekarski. 2018; 44 (261): 97–100. Available at: https://www.researchgate.net/publication/324130127_Danger_theory_as_a_common_mechanism_of_sarcoidosis_induction_by_infectious_and_non-infectious_factors_-_a_role_of_genetics_factors

16. Jouni H., Chareonthaitawee P. Unraveling inflammation and oxidative stress in cardiac sarcoidosis. Circ. Cardiovasc. Imaging. 2017; 10 (12): e007287. DOI: 10.1161/CIRCIMAGING.117.007287.

17. Ruža I., Lucāne Z. Serum and urinary calcium level in Latvian patients with sarcoidosis. Reumatologia. 2018; 56 (6): 377–381. DOI: 10.5114/reum.2018.80715.

18. Suzuki T., Tsushima K., Kawata N. et al. Estimation using the impulse oscillation system in patients with pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2015; 32 (2): 144–150. Available at: https://www.researchgate.net/publication/280876634_Estimation_Using_The_Impulse_Oscillation_System_In_Patients_With_Pulmonary_Sarcoidosis

19. Evfim’evskiy V.P., Borisov S.E., Bogorodskaya E.M. [Respiratory disorders in granulomatosis and common lesions of a different nature: a guide for physicians]. Moscow; 1998 (in Russian).

20. Van Schalkwyk E.M., Bezuidenhout J., Wyser C.P. et al. Comparison of bronchoalveolar lavage, open lung biopsy and lung function in sarcoidosis. Eur. Resp. J. 1997; 10 (Suppl. 25): 206s. Available at: https://eurekamag.com/research/030/633/030633831.php

21. Ungprasert P., Matteson E.L. Neurosarcoidosis. Rheum. Dis. Clin. North Am. 2017; 43 (4): 593–606. DOI: 10.1016/j.rdc.2017.06.008.

22. Duong H., Bonham C.A. Sarcoidosis-associated pulmonary hypertension: pathophysiology, diagnosis, and treatment. Clin. Pulm. Med. 2018; 25 (2): 52–60. DOI: 10.1097/CPM.0000000000000252.

23. Rusakov N.V., Mukhin N.A., Briko N.I. et al. Features of prevalence of sarcoidosis among the inhabitants of Moscow]. Gigiena i sanitariya. 2012; (4): 16–18. Available at: https://cyberleninka.ru/article/n/osobennosti-rasprostraneniya-sarkoidoza-v-usloviyah-moskvy?ysclid=l93wqbcyxo307456749 (in Russian).

24. Baranova O.P., Refitskaya N.V., Stepanenko T.A. et al. [Epidemiology of respiratory sarcoidosis in St. Petersburg (1998–2008)]. In: Chuchalin A.G., ed. [XIX National congress on respiratory diseases: collection of works]. Moscow: DesignPress; 2009: 216–217 (in Russian).

25. Petrov D.V., Ovsyannikov N.V., Konenko A.Yu. et al. [Result of the introduction of “Order of medical care of sarcoidosis patients” in Omsk city]. Bulletin of modern clinical medicine. 2013; 6 (2): 42–46. Available at: https://cyberleninka.ru/article/n/rezultaty-vnedreniya-poryadka-okazaniya-meditsinskoy-pomoschi-bolnym-sarkoidozom-v-gorode-omske/viewer (in Russian).

26. Vizel’ I.Ju., Vizel’ A.A [Characteristic of sarcoidosis register in the republic of Tatarstan]. Vestnik sovremennoy klinicheskoy mediciny. 2015; 8 (5): 18–26. Available at: http://vskmjournal.org/images/Files/Issues_Archive/2015/Issue_5/VSKM_2015_N_5_p18-26.pdf (in Russian).

27. Kryukov E.V., Antipushina D.N., Zaitsev A.A. [Sarcoidosis is an actual problem of various law enforcement agencies]. Vestnik Rossiyskoy voenno-medicinskoy akademii. 2016; 56 (4): 224–227 (in Russian).

28. Baughman R.P., Field S., Costabel U. et al. Sarcoidosis in America. Analysis based on health care use. Ann. Am. Thorac. Soc. 2016; 13 (8): 1244–1252. DOI: 10.1513/annalsats.201511-760oc.

29. Thomeer M., Demedts M., Vandeurzen K. et al. Registration of interstitial lung diseases by 20 centres of respiratory medicine in Flanders. Acta Clin. Belg. 2001; 56 (3): 163–172. DOI: 10.1179/acb.2001.026.

30. Morimoto T., Azuma A., Abe S. et al. Epidemiology of sarcoidosis in Japan. Eur. Respir. J. 2008; 31 (2): 372–379. DOI: 10.1183/09031936.00075307.

31. Hoffman A.L., Milman N., Byg K.E. Childhood sarcoidosis in Denmark 1979–1994: incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr. 2004; 93 (1): 30–36. DOI: 10.1111/j.1651-2227.2004.tb00670.x.

32. Terwiel M., van Moorsel C.H.M. Clinical epidemiology of familial sarcoidosis: a systematic literature review. Respir. Med. 2019; 149: 36–41. DOI: 10.1016/j.rmed.2018.11.022.

33. Vizel’ I.Yu., Shmelev E.I., Vizel’ A.A., Ganibaeva G.S. [Comparison of newly diagnosed patients with sarcoidosis of young and older age]. Russkiy meditsinskiy zhurnal. 2018; 10 (1):16–20. Available at: https://www.rusmedreview.com/articles/bolezni_dykhatelnykh_putey/Sravnenie_vnovy_vyyavlennyh_bolynyh_sarkoidozom_molodogo_istarshego_vozrasta/ (in Russian).

34. Terpigorev S.A., El Zein B.A., Vereshchagina V.M., Paleev N.R. [Sarcoidosis and problems of its classification]. Vestnik RAMN. 2012; (5): 30–37. Available at: https://cyberleninka.ru/article/n/sarkoidoz-i-problemy-ego-klassifikatsii/viewer (in Russian).

35. Bahmer T., Watz H., Develaska M. et al. Physical activity and fatigue in patients with sarcoidosis. Respiration. 2018; 95 (1): 18–26. DOI: 10.1159/000481827.

36. Il’kovich M.M., Baranova O.P. [Sarcoidosis of the respiratory system]. In: Il’kovich M.M., ed. [Interstitial and orphan lung diseases]. Moscow: GEOTAR-Media; 2019: 163–234 (in Russian).

37. Rabukhin A.E., Dobrokhotova M.N., Tonitrova N.S. [Sarcoidosis]. Moscow: Meditsina; 1975 (in Russian).

38. Wanat K.A., Rosenbach M. Cutaneous sarcoidosis. Clin. Chest Med. 2015; 36 (4): 685–702. DOI: 10.1016/j.ccm.2015.08.010.

39. Matsou A., Tsaousis K.T. Management of chronic ocular sarcoidosis: challenges and solutions. Clin. Ophthalmol. 2018; 12: 519–532. DOI: 10.2147/OPTH.S128949.

40. Judson M.A. The clinical features of sarcoidosis: a comprehensive review. Clin. Rev. Allergy Immunol. 2015; 49 (1): 63–78. DOI: 10.1007/s12016-014-8450-y.

41. Madaule S., Lauque D., Sailler L. et al. [Splenomegaly in sarcoidosis: clinical features and outcome. Analysis of 17 cases]. Rev. Med. Interne. 2004; 25 (5): 348–356. DOI: 10.1016/j.revmed.2003.11.007 (in French).

42. Löffler C., Bergner R. [Sarcoidosis: renal manifestations]. Z. Rheumatol. 2017; 76 (5): 398–407. DOI: 10.1007/s00393-017-0301-9 (in German).

43. Bechman K., Christidis D., Walsh S. et al. A review of the musculo-skeletal manifestations of sarcoidosis. Rheumatology (Oxford). 2018; 57 (5): 777–783. DOI: 10.1093/rheumatology/kex317.

44. Chapman M.N., Fujita A., Sung E.K. et al. Sarcoidosis in the head and neck: an illustrative review of clinical presentations and imaging findings. AJR Am. J. Roentgenol. 2017; 208 (1): 66–75. DOI: 10.2214/AJR.16.16058.

45. Ivanova D.A., Borisov S.E., Nedostup A.V., Pasha S. [Heart damage in sarcoidosis: diagnosis of latent and clinically manifested forms]. Vrach. 2008; (10): 28–33. Available at: https://www.elibrary.ru/download/elibrary_12924028_48123605.pdf (in Russian).

46. Dubrey S., Sharma R., Underwood R. et al. Sarcoidosis of the cardio-pulmonary systems. Clin. Med. (Lond.). 2016; 16 (1): 34–41. DOI: 10.7861/clinmedicine.16-1-34.

47. Suslina Z.A., Kistenev B.A., Maksimova M.Yu., Morgunov V.A. [Neurosarcoidosis]. Moscow: MEDpress-inform; 2009 (in Russian).

48. Ibitoye R.T., Wilkins A., Scolding N.J. Neurosarcoidosis: a clinical approach to diagnosis and management. J. Neurol. 2017; 264 (5): 1023–1028. DOI: 10.1007/s00415-016-8336-4.

49. Stern B.J., Royal W. 3rd, Gelfand J.M. et al. Definition and consensus diagnostic criteria for neurosarcoidosis: from the neurosarcoidosis Consortium Consensus Group. JAMA Neurol. 2018; 75 (12): 1546–1553. DOI: 10.1001/jamaneurol.2018.2295.

50. Block N.L., Kava B.R. Genitourinary sarcoidosis: an essential review for the practicing clinician. Indian J. Urol. 2017; 33 (1): 6–12. DOI: 10.4103/0970-1591.195724.

51. Ghrenassia E., Mekinian A., Chapelon-Albric C. et al. Digestive-tract sarcoidosis: French nationwide case-control study of 25 cases. Medicine (Baltimore). 2016; 95 (29): e4279. DOI: 10.1097/md.0000000000004279.

52. Sahu P., Sharma S., Sharma N. et al. Unusual clinical presentations in early-onset childhood sarcoidosis: a correlation or coincidence? J. Clin. Diagn. Res. 2017; 11 (8): WD01–03. DOI: 10.7860/JCDR/2017/27841.10389.

53. Fretzayas A., Moustaki M., Vougiouka O. The puzzling clinical spectrum and course of juvenile sarcoidosis. World J. Pediatr. 2011; 7 (2): 103–110. DOI: 10.1007/s12519-011-0261-0.

54. Nikolaev A.V. [Comparative assessment of indicators of nonspecific, cellular and humoral immunity in patients with tuberculosis and pulmonary sarcoidosis]. Vestnik Novgorodskogo gosudarstvennogo universiteta. 2019; 115 (3): 36–41. DOI: 10.34680/2076-8052.2019.3(115).36-41 (in Russian).

55. Hoffmann A. L., Milman N., Byg K. E. Childhood sarcoidosis in Denmark 1979–1994: incidence, clinical features and laboratory results at presentation in 48 children. Acta Paediatr. 2004; 93 (1): 30–36. DOI: 10.1080/08035250310007213.

56. Kempisty A., Białas-Chromiec B., Borkowska D., Kuś J. Interferon gamma release assays based on M. tuberculosis-specific antigens in sarcoidosis patients. Pneumonol. Alergol. Pol. 2015; 83 (2): 126–134. DOI: 10.5603/PiAP.2015.0020.

57. Gupta D., Kumar S., Aggarwal A.N. et al. Interferon gamma release assay (QuantiFERON-TB Gold in Tube) in patients of sarcoidosis from a population with high prevalence of tuberculosis infection. Sarcoidosis Vasc. Diffuse Lung Dis. 2011; 28 (2): 95–101. Available at: https://www.researchgate.net/publication/51834535_Interferon_gamma_release_assay_QuantiFERON-TB_Gold_In_Tube_in_patients_of_sarcoidosis_from_a_population_with_high_prevalence_of_tuberculosis_infection

58. Nosal A., Schleissner L.A., Mishkin F.S., Lieberman J. Angiotensin-I-converting enzyme and gallium scan in noninvasive evaluation of sarcoidosis. Ann. Intern. Med. 1979; 90 (3): 328–331. DOI: 10.7326/0003-4819-90-3-328.

59. Baughman R.P., Shipley R., Eisentrout C.E. Predictive value of gallium scan, angiotensin-converting enzyme level, and bronchoalveolar lavage in two-year follow-up of pulmonary sarcoidosis. Lung. 1987; 165 (6): 371–377. DOI: 10.1007/bf02714452.

60. Abe S., Munakata M., Nishimura M. et al. Gallium-67 scintigraphy, bronchoalveolar lavage, and pathologic changes in patients with pulmonary sarcoidosis. Chest. 1984; 85 (5): 650–655. DOI: 10.1378/chest.85.5.650.

61. Okayama K., Kurata C., Tawarahara K. et al. Diagnostic and prognostic value of myocardial scintigraphy with thallium-201 and gallium-67 in cardiac sarcoidosis. Chest. 1995; 107 (2): 330–334. DOI: 10.1378/chest.107.2.330.

62. Bekerman C., Szidon J. P., Pinsky S. The role of gallium-67 in the clinical evaluation of sarcoidosis. Semin. Roentgenol. 1985; 20 (4): 400–409. DOI: 10.1016/0037-198x(85)90047-1.

63. Jerusalem G., Beguin Y., Fassotte M.F. et al. Whole-body positron emission tomography using18F-fluorodeoxyglucose for posttreatment evaluation in Hodgkin’s disease and non-Hodgkin’s lymphoma has higher diagnostic and prognostic value than classical computed tomography scan imaging. Blood. 1999; 94 (2): 429–433. Available at: https://www.researchgate.net/publication/12077850_Whole-body_positron_emission_tomography_using_18F-fluorodeoxyglucose_compared_to_standard_procedures_for_staging_patients_with_Hodgkins_disease

64. Ozer O., Eskazan A.E., Ar M.C. et al. Sarcoidosis mimicking lymphoma on positron emission tomography-computed tomography in two patients treated for lymphoma: two case reports. J. Med. Case Rep. 2009; 3: 7306. DOI: 10.4076/1752-1947-3-7306.

65. Dziedzic D.A., Peryt A., Orlowski T. The role of EBUS-TBNA and standard bronchoscopic modalities in the diagnosis of sarcoidosis. Clin. Respir. J. 2017; 11 (1): 58–63. DOI: 10.1111/crj.12304.

66. Millward K., Fiddler C.A., Thillai M. Update on sarcoidosis guidelines. Curr. Opin. Pulm. Med. 2021; 27 (5): 484–489. DOI: 10.1097/MCP.0000000000000807.

67. Demir O.F., Onal O. Is mediastinoscopy an effective diagnostic method in mediastinal area evaluation in pediatric patients? Asian. J. Surg. 2020; 43 (6): 690–695. DOI: 10.1016/j.asjsur.2019.09.012.

68. Yoon H.Y., Kim H.M., Kim Y.J., Song J.W. Prevalence and incidence of sarcoidosis in Korea: a nationwide population-based study. Respir. Res. 2018; 19 (1): 158. DOI: 10.1186/s12931-018-0871-3.

69. Verleden S.E., Vanstapel A., De Sadeleer L. et al. Distinct airway involvement in subtypes of end-stage fibrotic pulmonary sarcoidosis. Chest. 2021; 160 (2): 562–571. DOI: 10.1016/j.chest.2021.01.003.

70. Khan T., Selvakumar D., Trivedi S. et al. The value of endomyocardial biopsy in diagnosis and guiding therapy. Pathology. 2017; 49 (7): 750–756. DOI: 10.1016/j.pathol.2017.08.004.

71. Zhang C., Chan K.M., Schmidt L.A., Myers J.L. Histopathology of explanted lungs from patients with a diagnosis of pulmonary sarcoidosis. Chest. 2016; 149 (2): 499–507. DOI: 10.1378/chest.15-0615.

72. Buxbaum J., Papademetriou M., Klipfel N. et al. Biliary sarcoidosis: early diagnosis minimizes the need for surgery. Am. J. Respir. Crit. Care Med. 2013; 187 (5): 556–559. DOI: 10.1164/ajrccm.187.5.556.

73. Siavelis H.A., Herrmann M.E., Aranha G.V. et al. Sarcoidosis and the pancreas. Surgery. 1999; 125 (4): 456–461. DOI: 10.1016/S0039-6060(99)70014-0.

74. Manchanda A., Patel S., Jiang J.J., Babu A.R. Thyroid: an unusual hideout for sarcoidosis. Endocr. Pract. 2013; 19 (2): e40–43. DOI: 10.4158/EP12131.CR.

75. James D.G., Jones Williams W., eds. Sarcoidosis and other granulomatous disorders. Philadelphia: Saunders; 1985: 163–166. DOI: 10.1002/PPUL.1950010513.

76. Shmelev E.I. [Differential diagnosis of disseminated diseases of the lungs of a non-tumor nature]. Russkiy meditsinskiy zhurnal. 2001; (21): 918–922. Available at: https://www.rmj.ru/articles/bolezni_dykhatelnykh_putey/Differencialynaya_diagnostika_disseminirovannyh_zabolevaniy_legkih_neopuholevoy_prirody/ (in Russian).

77. El Sayed F., Torbey G., Youssef H., Chababi M. Childhood sarcoidosis: diagnostic issues. Dermatol. Online J. 2013; 19 (10): 20037. DOI: 10.5070/D31910020037.

78. Deverrière G., Flamans-Klein A., Firmin D. et al. [Early onset pediatric sarcoidosis, diagnostic problems]. Arch. Pediatr. 2012; 19 (7): 707–710. DOI: 10.1016/j.arcped.2012.04.024.

79. Bradley B., Branley H.M., Egan J.J. et al. 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: v1–58. DOI: 10.1136/thx.2008.101691.

80. Baughman R., Drent M., Judson M. et al. Sarcoidosis treatment guidelines. Available at: https://stopsarcoidosis.org/wp-content/uploads/2013/03/FSR-Physicians-Protocol1.pdf

81. Balasubramanian A., Wade S.W., Adler R.A. et al. Glucocorticoid exposure and fracture risk in a cohort of US patients with selected conditions. J. Bone Miner Res. 2018; 33 (10): 1881–1888. DOI: 10.1002/jbmr.3523.

82. James W.E., Baughman R. Treatment of sarcoidosis: grading the evidence. Expert Rev. Clin. Pharmacol. 2018; 11 (7): 677–687. DOI: 10.1080/17512433.2018.1486706.

83. Baughman R.P., Nunes H., Sweiss N.J. et al. Established and experimental medical therapy of pulmonary sarcoidosis. Eur. Respir. J. 2013; 41 (6): 1424–1438. DOI: 10.1183/09031936.00060612.

84. Soto-Gomez N., Peters J.I., Nambiar A.M. Diagnosis and management of sarcoidosis. Am. Fam. Physician. 2016; 93 (10): 840–850. https://www.aafp.org/pubs/afp/issues/2016/0515/p840.html

85. Vizel’ A. A., Kalver D. A., Vizel’ I. Ju. et al. [Assessment of the effect of alpha-tocopherol on the course of newly diagnosed pulmonary sarcoidosis: a comparative study]. Tuberkulyoz i bolezni lyogkih. 2020; 98 (10): 33–40. DOI: 10.21292/2075-1230-2020-98-10-33-40 (in Russian).

86. Kostina Z.I. [Results of treatment of sarcoidosis of the lungs]. Problemy tuberkuleza. 1977; (8): 44–50 (in Russian).

87. Diallo B.D., Diot B., Flament T. et al. [The pentoxifylline, a corticosteroid sparing in the treatment of sarcoidosis: a case report]. Rev. Pneumol. Clin. 2018; 74 (1): 48–51. DOI: 10.1016/j.pneumo.2017.08.014.

88. Refiskaya N.V., Stepanenko T.A., Jablonskiy P.K., Baranova O.P. The use of tumor necrosis factor alpha inhibitor pentoxifylline in the treatment of patients with I and II stages of pulmonary sarcoidosis. Eur. Respir. J. 2006; 28 (Suppl. 50): E3134. Available at: https://www.ers-education.org/lr/show-details/?idP=8337

89. Vizel A.A., Vizel E.A., Nasretdinova G.R. et al. [Effectiveness of low-dose pentoxithylline combined with tocopherol acetate in intrathoracic sacroidosis]. Pul’monologiya. 2005; (1): 24–28. DOI: 10.18093/0869-0189-2005-0-1-24-28 (in Russian).

90. Crommelin H.A., Vorselaars A.D., van Moorsel C.H. et al. Anti-TNF therapeutics for the treatment of sarcoidosis. Immunotherapy. 2014; 6 (10): 1127–1143. DOI: 10.2217/imt.14.65.

91. About improvement of the sanatorium-resort and rehabilitation help to patients with tuberculosis. Order of the Ministry of Health and Medical Industry of the Russian Federation No.291 (July 19, 1996)]. Available at: https://tkrfkod.ru/zakonodatelstvo/prikaz-minzdravmedproma-rf-ot-19071996-n-291/ (in Russian).

92. Vizel’ A.A., Guryleva M.Ye. [Sarcoidosis in children]. Pediatriya. Zhurnal imeni G.N.Speranskogo. 2004; 83 (2): 62–66. Available at: https://pediatriajournal.ru/files/upload/mags/263/2004_2_1177.pdf (in Russian).

93. Paramothayan N., Lasserson T., Jones P. Corticosteroids for pulmonary sarcoidosis. Cochrane Database Syst. Rev. 2005; (2): CD001114. DOI: 10.1002/14651858.cd001114.pub2.

94. State Register of Medicines. Prednisolone. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=5bb36449-e307-43d1-ad5a-7171d992c66a&t= (in Russian).

95. Milman N., Hoffmann A. L., Byg K. E. Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis. Acta Paediatrica.1998; 87 (8): 871–878. DOI: 10.1080/080352598750013662.

96. Adami G., Saag K.G. Glucocorticoid-induced osteoporosis: 2019 concise clinical review. Osteoporos Int. 2019; 30 (6): 1145–1156. DOI: 10.1007/s00198-019-04906-x.

97. Scherholz M.L., Schlesinger N., Androulakis I.P. Chronopharmacology of glucocorticoids. Adv. Drug Deliv. Rev. 2019. 151–152; 245–261. DOI: 10.1016/j.addr.2019.02.004.

98. Dinsen S., Baslund B., Klose M. et al. Why glucocorticoid withdrawal may sometimes be as dangerous as the treatment itself. Eur. J. Intern. Med. 2013; 24 (8): 714–720. DOI: 10.1016/j.ejim.2013.05.014.

99. Coker R. K. Guidelines for the use of corticosteroids in the treatment of pulmonary sarcoidosis. Drugs. 2007; 67 (8): 1139–1147. DOI: 10.2165/00003495-200767080-00004.

100. Herlow L.B., Rasmussen N. [Laryngeal sarcoidosis in 13 year-old teenager]. Ugeskr. Laeger. 2010; 172 (45): 3116–3117. Available at: https://ugeskriftet.dk/files/scientific_article_files/2014-04//artikel_8769.pdf (in Danish).

101. State Register of Medicines. Methotrexate. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=43da4be2-b2bc-4eaa-8031-aae6250bf870&t= (in Russian).

102. Cremers J.P., Drent M., Bast A. 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 (5): 545–561. DOI: 10.1097/mcp.0b013e3283642a7a.

103. Gavrysyuk V., Merenkova E., Gumeniuk G. et al. [Effectiveness and safety of methotrexate monotherapy in patients with pulmonary sarcoidosis]. Georgian Med. News. 2018; (283): 34–38. Available at: https://geomednews.com/s/480918712df344a4a77508d4cd7815ab/files/uploaded/V283_N10_October_2018.pdf? (in Russian).

104. Baughman R.P., Winget D.B., Lower E.E. Methotrexate is steroid sparing in acute sarcoidosis: results of a double blind, randomized trial. Sarcoidosis Vasc. Diffuse Lung Dis. 2000; 17 (1): 60–66. Available at: https://read.qxmd.com/read/10746262/methotrexate-is-steroid-sparing-in-acute-sarcoidosis-results-of-a-double-blind-randomized-trial

105. Vizel’ A.A., Vizel’ I.Ju. [The use of methotrexate in sarcoidosis (according to a controlled prospective study)]. Klinicheskaya meditsina. 2015; 93 (1): 41–46. Available at: https://cyberleninka.ru/article/n/primenenie-metotreksata-pri-sarkoidoze-po-dannym-kontroliruemogo-prospektivnogo-issledovaniya/viewer (in Russian).

106. Gedalia A., Molina J.F., Ellis G.S. Jr. et al. Low-dose methotrexate therapy for childhood sarcoidosis. J. Pediatr. 1997; 130 (1): 25–29. DOI: 10.1016/s0022-3476(97)70306-8.

107. Gedalia A., Khan T.A., Shetty A.K. et al. Childhood sarcoidosis: Louisiana experience. Clin. Rheumatol. 2016; 35 (7): 1879–1884. DOI: 10.1007/s10067-015-2870-9.

108. Birnie D., Beanlands R.S.B., Nery P. et al. Cardiac sarcoidosis multi-center randomized controlled trial (CHASM CS-RCT). Am. Heart J. 2020; 220: 246–252. DOI: 10.1016/j.ahj.2019.10.003.

109. Ning N., Guo H.H., Iagaru A. et al. Serial cardiac FDG-PET for the diagnosis and therapeutic guidance of patients with cardiac sarcoidosis. J. Card. Fail. 2019; 25 (4): 307–311. DOI: 10.1016/j.cardfail.2019.02.018.

110. Soriano F. G., Caramelli P., Nitrini R., Rocha A.S. Neurosarcoidosis: therapeutic success with methotrexate. Postgrad. Med. J. 1990; 66 (772): 142–143. DOI: 10.1136/pgmj.66.772.142.

111. Judson M. A. Sarcoidosis: clinical presentation, diagnosis, and approach to treatment. Am. J. Med. Sci. 2008; 335 (1): 26–33. DOI: 10.1097/maj.0b013e31815d8276.

112. Vorselaars A.D., Cremers J.P., Grutters J.C., Drent M. Cytotoxic agents in sarcoidosis: which one should we choose? Curr. Opin. Pulm. Med. 2014; 20 (5): 479–487. DOI: 10.1097/mcp.0000000000000078.

113. Ballul T., Borie R., Crestani B. et al. Treatment of cardiac sarcoidosis: a comparative study of steroids and steroids plus immunosuppressive drugs. Int. J. Cardiol. 2019; 276: 208–211. DOI: 10.1016/j.ijcard.2018.11.131.

114. Lewis S.J., Ainslie G.M., Bateman E.D. Efficacy of azathioprine as second-line treatment in pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 1999; 16 (1): 87–92. Available at: https://www.semanticscholar.org/paper/Efficacy-of-azathioprine-as-second-line-treatment-Lewis-Ainslie/f8f6fe3938be432c3d49af53d4d8fcacac308453

115. Beegle S.H., Barba K., Gobunsuy R., Judson M.A. Current and emerging pharmacological treatments for sarcoidosis: a review. Drug Des. Devel. Ther. 2013; 7: 325–328. DOI: 10.2147/dddt.s31064.

116. State Register of Medicines. Azathioprine. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=186056b6-254d-44c1-a1c8-aac-7f595a198&t= (in Russian).

117. El Jammal T., Jamilloux Y., Gerfaud-Valentin M. et al. Refractory sarcoidosis: a review. Ther. Clin. Risk. Manag. 2020; 16: 323–345. DOI: 10.2147/TCRM.S192922.

118. Baughman R.P., Lower E.E. Leflunomide for chronic sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2004; 21 (1): 43–48. DOI: 10.1007/s11083-004-5178-y.

119. Raj R., Nugent K. Leflunomide-induced interstitial lung disease (a systematic review). Sarcoidosis Vasc. Diffuse Lung Dis. 2013; 30 (3): 167–176. Available at: https://www.researchgate.net/publication/258957656_Leflunomide-induced_interstitial_lung_disease_a_systematic_review

120. Bohelay G., Bouaziz J.D., Nunes H. et al. Striking leflunomide efficacy against refractory cutaneous sarcoidosis. J. Am. Acad. Dermatol. 2014; 70 (5): e111–113. DOI: 10.1016/j.jaad.2013.10.048.

121. Sahoo D.H., Bandyopadhyay D., Xu M. et al. Effectiveness and safety of leflunomide for pulmonary and extrapulmonary sarcoidosis. Eur. Respir. J. 2011; 38 (5): 1145–1150. DOI: 10.1183/09031936.00195010.

122. State Register of Medicines. Arresto. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=46c1c8dd-0c2e-48b0-bf3e-be5a206a1238&t= (in Russian).

123. Hamzeh N., Voelker A., Forssen A. et al. Efficacy of mycophenolate mofetil in sarcoidosis. Respir. Med. 2014; 108 (11): 1663–1669. DOI: 10.1016/j.rmed.2014.09.013.

124. Muhin N.A., ed. [Sarcoidosis: clinical guidelines]. Moscow: IMA-PRESS; 2009 (in Russian).

125. Kikuchi N., Nunoda S., Serizawa N. et al. Combination therapy with corticosteroid and mycophenolate mofetil in a case of refractory cardiac sarcoidosis. J. Cardiol. Cases. 2016; 13 (4): 125–128. DOI: 10.1016/j.jccase.2015.12.008.

126. State Register of Medicines. Flomiren. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=9fd35729-ab22-4b2c-a80d-41619164b8b2&t= (in Russian).

127. Moudgil A., Przygodzki R. M., Kher K. K. Successful steroid-sparing treatment of renal limited sarcoidosis with mycophenolate mofetil. Pediatr. Nephrol. 2006; 21 (2): 281–285. DOI: 10.1007/s00467-005-2086-3.

128. Judson M. A. Sarcoidosis: clinical presentation, diagnosis, and approach to treatment. Am. J. Med. Sci. 2008; 335 (1): 26–33. DOI: 10.1097/maj.0b013e31815d8276.

129. State Register of Medicines. Endoxan. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=cc8c311d-6bae-4a30-b1d2-f4e74e5f3644&t (in Russian).

130. Rabinowitz M.P., Murchison A.P. Orbital sarcoidosis treated with hydroxychloroquine. Orbit. 2011; 30 (1): 13–15. DOI: 10.3109/01676830.2010.524268.

131. Kiedrowicz M., Kacalak-Rzepka A., Bielecka-Grzela S., Maleszka R. [Antimalarial drugs in contemporary dermatologic therapy]. Ann. Acad. Med. Stetin. 2011; 57 (1): 38–44. Available at: https://www.researchgate.net/publication/224975968_Antimalarial_drugs_in_contemporary_dermatologic_therapy

132. State Register of Medicines. Plaquenil. Available at: https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=92eff6a5-c78c-4d28-910b-cdc-c78986307&t= (in Russian).

133. Baltzan M., Mehta S., Kirkham T.H., Cosio M.G. Randomized trial of prolonged chloroquine therapy in advanced pulmonary sarcoidosis. Am. J. Respir. Crit. Care Med. 1999; 160 (1): 192–197. DOI: 10.1164/ajrccm.160.1.9809024.

134. Zabel P., Entzian P., Dalhoff K., Schlaak M. Pentoxifylline in treatment of sarcoidosis. Am. J. Respir. Crit. Care Med. 1997; 155 (5): 1665–1669. DOI: 10.1164/ajrccm.155.5.9154873.

135. Park M.K., Fontana Jr., Babaali H. et al. Steroid-sparing effects of pentoxifylline in pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2009; 26 (2): 121–131. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2946799/

136. Popova E.N., Bolevich S.B., Fomin V.V. et al. Pulmonary hypertension associated with sarcoidosis and experience of treatment with pentoxifylline (Vasonite). Eur. Respir. J. 2006; 28 (Suppl. 50): 2491. Available at: https://www.ers-education.org/lr/show-details/?idP=7643

137. Borodina G.L. [Anticytokine therapy with pentoxifylline as monotherapy and in combination with glucocorticosteroids in respiratory sarcoidosis]. Tuberkulez i bolezni legkikh. 2013; (8): 15–20. Available at: https://www.elibrary.ru/download/elibrary_20658497_99153726.pdf (in Russian).

138. Kostina Z.I., Brazhenko N.A., Nasorina R.N. [Long-term results of observation of treated patients with respiratory sarcoidosis]. Problemy tuberkuleza. 1995; (3): 34–37 (in Russian).

139. Zubovich G.L., Abramovskaya A.K., Kamyshnikov V.S. et al. [Homeostatic indices in pulmonary sarcoidosis]. Pul’monologiya. 1996; (2): 50–54. Available at: https://journal.pulmonology.ru/pulm/article/view/3430/2817 (in Russian).

140. Drent M., Cremers J.P., Jansen T.L., Baughman R.P. Practical eminence and experiencebased recommendations for use of TNF-alpha inhibitors in sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2014; 31 (2): 91–107. Available at: https://www.wasog.org/dynamic/media/78/documents/2014_Drent_et_al_Sarcoidosis_anti-TNF.pdf

141. Simonini G., Taddio A., Cattalini M. et al. Prevention of flare recurrences in childhood-refractory chronic uveitis: an open-label comparative study of adalimumab versus infliximab. Arthritis Care Res. (Hoboken). 2011; 63 (4): 612–618. DOI: 10.1002/acr.20404.

142. Orandi A.B., Eutsler E., Ferguson C. et al. Sarcoidosis presenting as granulomatous myositis in a 16-year-old adolescent. Pediatr. Rheumatol. Online J. 2016; 14 (1): 59. DOI: 10.1186/s12969-016-0121-5.

143. Judson M.A., Baughman R.P., Costabel U. et al. Safety and efficacy of ustekinumab or golimumab in patients with chronic sarcoidosis. Eur. Respir. J. 2014; 44 (5): 1296–1307. DOI: 10.1183/09031936.00000914.

144. Utz J.P., Limper A.H., Kalra S. et al. Etanercept for the treatment of stage II and III progressive pulmonary sarcoidosis. Chest. 2003; 124 (1): 177–185. DOI: 10.1378/chest.124.1.177.

145. Majjad A., Bezza A., Biyi A. et al. Pulmonary sarcoidosis following etanercept treatment for ankylosing spondylitis: a case report and review of the literature. Case Rep. Rheumatol. 2018; 2018: 9867248. DOI: 10.1155/2018/9867248.

146. Kim T.K., Kang S.H., Moon H.S. et al. Pulmonary sarcoidosis that developed during the treatment of a patient with Crohn disease by using infliximab. Ann. Coloproctol. 2017; 33 (2): 74–77. DOI: 10.3393/ac.2017.33.2.74.

147. Instructions for the medical preparation Vargatef. Approval date of the Ministry Health of the Russian Federation July 10, 2020. Available at: https://www.boehringer-ingelheim.ru/sites/ru/files/files/vargatef_pil_10-07-2020.pdf (in Russian).

148. Flaherty K.R., Wells A. U., Cottin V. et al. Nintedanib in progressive fibrosing interstitial lung diseases. N. Engl. J. Med. 2019; 381 (18): 1718–1727. DOI: 10.1056/NEJMoa1908681.

149. Shmelev E.I. [Sarcoidosis]. Pul’monologiya i allergologiya. 2004; 13 (2): 3–10. Available at: https://atmosphere-ph.ru/modules/Magazines/articles/pulmo/ap_2_2004_03.pdf (in Russian).

150. Romanov V.V. Extracorporeal methods in the treatment of patients with sarcoidosis. Problemy tuberkuleza. 2001; (3): 45–49 (in Russian).

151. Baranova O.P., Baklanova O.E., Voinov V.A., Karchevsky K.S. [The use of plasmapheresis in the complex treatment of patients with pulmonary sarcoidosis]. In: Chuchalin A.G., ed. [XIX National Congress on respiratory diseases: collection of works]. Moscow: DesignPress, 2009: 215 (in Russian).

152. Smelaya T.V., Sivtsevich N.N., Zholobov I.M. [Extracorporeal pharmacotherapy for treatment of patients with various nosologies]. Meditsinskiy vestnik MVD. 2017; 91 (6): 44–50 (in Russian).

153. Taimeh Z., Hertz M.I., Shumway S., Pritzker M. Lung transplantation for pulmonary sarcoidosis. Twenty-five years of experience in the USA. Thorax. 2016; 71 (4): 378–379. DOI: 10.1136/thorax-jnl-2015-207497.

154. Salamo O., Roghaee S., Schweitzer M.D. et al. White donor, younger donor and double lung transplant are associated with better survival in sarcoidosis patients. Sci. Rep. 2018; 8 (1): 6968. DOI: 10.1038/s41598-018-25144-x.

155. Meyer K.C. Lung transplantation for pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 2019; 36 (2): 92–107. DOI: 10.36141/svdld.v36i2.7163.

156. Rosenthal D.G., Anderson M.E., Petek B.J. et al. Invasive hemodynamics and rejection rates in patients with cardiac sarcoidosis after heart transplantation. Can. J. Cardiol. 2018; 34 (8): 978–982. DOI: 10.1016/j.cjca.2018.03.021.

157. Sawahata M., Shijubo N., Johkoh T. et al. Progression of central-peripheral band and traction bronchiectasis clusters leading to chronic respiratory failure in a patient with fibrotic pulmonary sarcoidosis. Intern. Med. 2021; 60 (1): 111–116. DOI: 10.2169/internalmedicine.4862-20.

158. Faverio P., De Giacomi F., Sardella L. et al. Management of acute respiratory failure in interstitial lung diseases: overview and clinical insights. BMC Pulm. Med. 2018; 18 (1): 1–13. DOI: 10.1186/s12890-018-0643-3.

159. Avdeev S.N. [Pulmonary hypertension in sarcoidosis]. Pul’monologiya. 2016; 26 (6): 725–735. DOI: 10.18093/0869-0189-2016-26-6-725-735 (in Russian).

160. Parikh K.S., Dahhan T., Nicholl L. et al. Clinical features and outcomes of patients with sarcoidosis-associated pulmonary hypertension. Sci. Rep. 2019; 9 (1): 4061. DOI: 10.1038/s41598-019-40030-w.

161. Corte T.J., Wells A.U., Nicholson A.G. et al. Pulmonary hypertension in sarcoidosis: a review. Respirology. 2011; 16 (1): 69–77. DOI: 10.1111/j.1440-1843.2010.01872.x.

162. Borodina G.L. [Developing of medical rehabilitation program and evoluation of its efficiency in pulmonary sarcoidosis patients]. Vestnik sovremennoy klinicheskoy meditsiny. 2012; 5 (1): 11–20. Available at: http://vskmjournal.org/images/Files/Issues_Archive/2012/Issue_1/VSKM_2012_N_1_p11-20.pdf (in Russian).

163. Niedoszytko P. [Rehabilitation of patients with sarcoidosis]. Pol. Merkur. Lekarski. 2018; 44 (261): 150–151. Available at: https://www.researchgate.net/publication/324129986_Rehabilitation_of_patients_with_sarcoidosis

164. Chernikov A.Yu. [On the effectiveness of medical rehabilitation of patients with sarcoidosis in the general medical network]. Zdravookhranenie Rossiyskoy Federatsii. 2008; (4): 36–39 (in Russian).

165. Chernikov A.Yu., Daurov B.I. [Efficiency of clinical examination of patients with sarcoidosis in a general medical network]. Problemy tuberkulyoza i bolezney lyogkih. 2007; 84 (8): 3–5 (in Russian).

166. Garmash Yu.Yu. [Monitoring of patients with sarcoidosis: basic principles]. Rossiyskiy meditsinskiy zhurnal. 2009; (1): 22–23 (in Russian).