EUROPEAN RESPIRATORY SOCIETY PRACTICE GUIDELINES IN TREATMENT OF SARCOIDOSIS: CLINICAL APPROACH AND SUGGESTIONS
Abstract
Sarcoidosis can affect any organ and thus lead to a significant impairment of quality of life, even to an increase in mortality rate. Neurosarcoidosis, cardiac sarcoidosis, and pulmonary fibrosis in sarcoidosis are the forms of sarcoidosis with the highest mortality rate, and with the lowest response rate to current therapy. A group of experts in the field of sarcoidosis, supported by the European Respiratory Society, created practice guidelines on treatment of the most common forms of sarcoidosis, and provided suggestions for further research in order to create new therapeutic protocols. The evidence was obtained by reviewing literature. The aim of this paper is to bring these guidelines closer to doctors in daily clinical practice, in order to improve patients’ quality of life and reduce mortality from sarcoidosis.
References
1) Stjepanovic MI, Mihailovic-Vucinic V, Spasovski V, Milin-Lazovic J, Skodric-Trifunovic V, Stankovic S, et al. Genes and metabolic pathway of sarcoidosis: identification of key players and risk modifiers. Arch Med Sci. 2019 Sep;15(5):1138-46. doi: 10.5114/aoms.2018.79682.
2) Swigris JJ, Olson AL, Huie TJ, Fernandez-Perez ER, Solomon J, Sprunger D, et al. Sarcoidosis-related mortality in the United States from 1988 to 2007. Am J Respir Crit Care Med. 2011 Jun 1;183(11):1524-30. doi: 10.1164/rccm.201010-1679OC.
3) Baughman RP, Wells AU. Advanced sarcoidosis. Curr Opin Pulm Med 2019; 25: 497–504. doi: 10.1097/MCP.0000000000000612.
4) 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 Dec 16;58(6):2004079. doi: 10.1183/13993003.04079-2020.
5) Alonso-Coello P, Schünemann HJ, Moberg J, Brignardello-Petersen R, Akl EA, Davoli M, et al. GRADE Working Group. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 1: Introduction. BMJ. 2016 Jun 28;353:i2016. doi: 10.1136/bmj.i2016.
6) American Thoracic Society, European Respiratory Society (ERS) and World Association of Sarcoidosis and Other Granulomatous Disorders. 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: 736–55. doi: 10.1164/ajrccm.160.2.ats4-99.
7) Crouser ED, Maier LA, Wilson KC, Bonham CA, Morgenthau AS, Patterson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020 Apr 15;201(8):e26-e51. doi: 10.1164/rccm.202002-0251ST.
8) Gangemi AJ, Myers CN, Zheng M, Brown J, Butler-LeBair M, Cordova F, et al. Mortality for sarcoidosis patients on the transplant wait list in the Lung Allocation Score era: Experience from a high volume center. Respir Med. 2019 Oct;157:69-76. doi: 10.1016/j.rmed.2019.09.001.
9) Akashi H, Kato TS, Takayama H, Naka Y, Farr M, Mancini D, et al. Outcome of patients with cardiac sarcoidosis undergoing cardiac transplantation--single-center retrospective analysis. J Cardiol. 2012 Nov;60(5):407-10. doi: 10.1016/j.jjcc.2012.07.013.
10) Walsh SL, Wells AU, Sverzellati N, Keir GJ, Calandriello L, Antoniou KM, et al. An integrated clinicoradiological staging system for pulmonary sarcoidosis: a case-cohort study. Lancet Respir Med. 2014 Feb;2(2):123-30. doi: 10.1016/S2213-2600(13)70276-5.
11) Uzunhan Y, Nunes H, Jeny F, Lacroix M, Brun S, Brillet PY, et al. Chronic pulmonary aspergillosis complicating sarcoidosis. Eur Respir J. 2017 Jun 15;49(6):1602396. doi: 10.1183/13993003.02396-2016.
12) Huitema MP, Bakker ALM, Mager JJ, Rensing BJWM, Smits F, Snijder RJ, et al. Prevalence of pulmonary hypertension in pulmonary sarcoidosis: the first large European prospective study. Eur Respir J. 2019 Oct 31;54(4):1900897. doi: 10.1183/13993003.00897-2019.
13) Israel HL, Fouts DW, Beggs RA. A controlled trial of prednisone treatment of sarcoidosis. Am Rev Respir Dis 1973; 107: 609–14. doi: 10.1164/arrd.1973.107.4.609.
14) Broos CE, Poell LHC, Looman CWN, In 't Veen JCCM, Grootenboers MJJH, Heller R, van den Toorn LM, Wapenaar M, Hoogsteden HC, Kool M, Wijsenbeek MS, van den Blink B. No evidence found for an association between prednisone dose and FVC change in newly-treated pulmonary sarcoidosis. Respir Med. 2018 May;138S:S31-S37. doi: 10.1016/j.rmed.2017.10.022.
15) Milman N, Graudal N, Grode G, Munch E. No effect of high-dose inhaled steroids in pulmonary sarcoidosis: a double-blind, placebo-controlled study. J Intern Med. 1994 Sep;236(3):285-90. doi: 10.1111/j.1365-2796.1994.tb00798.x. PMID: 8077885.
16) Baughman RP, Winget DB, Lower EE. Methotrexate is steroid sparing in acute sarcoidosis: results of a double blind, randomized trial. Sarcoidosis Vasc Diffuse Lung Dis. 2000 Mar;17(1):60-6. PMID: 10746262.
17) Fang C, Zhang Q, Wang N, Jing X, Xu Z. Effectiveness and tolerability of methotrexate in pulmonary sarcoidosis: A single center real-world study. Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(3):217-27. doi: 10.36141/svdld.v36i3.8449.
18) Baughman RP, Drent M, Kavuru M, Judson MA, Costabel U, du Bois R, et al. Sarcoidosis Investigators. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med. 2006 Oct 1;174(7):795-802. doi: 10.1164/rccm.200603-402OC.
19) Stjepanovic M, Popevic S, Dimic Janjic S et al. Odabrana poglavlja iz pulmologije, Beograd: Medicinski fakultet Univerziteta, CIBID, 2023. p 159-66
20) Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. INBUILD Trial Investigators. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019 Oct 31;381(18):1718-27. doi: 10.1056/NEJMoa1908681.
21) Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, et al. Progressive pulmonary fibrosis: an expert group consensus statement. Eur Respir J. 2023 Mar 30;61(3):2103187. doi: 10.1183/13993003.03187-2021.
22) Matteson EL, Kelly C, Distler JHW, Hoffmann-Vold AM, Seibold JR, Mittoo S, et al. INBUILD Trial Investigators. Nintedanib in Patients With Autoimmune Disease-Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial. Arthritis Rheumatol. 2022 Jun;74(6):1039-47. doi: 10.1002/art.42075.
23) Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997; 336: 1224–34. doi: 10.1056/NEJM199704243361706.
24) Baughman RP, Lower EE. Evidence-based therapy for cutaneous sarcoidosis. Clin Dermatol 2007; 25: 334–40. doi: 10.1016/j.clindermatol.2007.03.011.
25) Stagaki E, Mountford WK, Lackland DT, Judson MA. The treatment of lupus pernio: results of 116 treatment courses in 54 patients. Chest. 2009 Feb;135(2):468-76. doi: 10.1378/chest.08-1347.
26) Baughman RP, Judson MA, Lower EE, Drent M, Costabel U, Flavin S, Lo KH, et al. Sarcoidosis Investigators. Infliximab for chronic cutaneous sarcoidosis: a subset analysis from a double-blind randomized clinical trial. Sarcoidosis Vasc Diffuse Lung Dis. 2016 Jan 15;32(4):289-95. PMID: 26847095.
27) Sakkat A, Cox G, Khalidi N, Larche M, Beattie K, Renzoni EA, et al. Infliximab therapy in refractory sarcoidosis: a multicenter real-world analysis. Respir Res. 2022 Mar 9;23(1):54. doi: 10.1186/s12931-022-01971-5.
28) Baughman RP, Teirstein AS, Judson MA, Rossman MD, Yeager H Jr, Bresnitz EA, et al. Case Control Etiologic Study of Sarcoidosis (ACCESS) research group. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med. 2001 Nov 15;164(10 Pt 1):1885-9. doi: 10.1164/ajrccm.164.10.2104046.
29) Ribeiro Neto ML, Jellis CL, Joyce E, Callahan TD, Hachamovitch R, Culver DA. Update in Cardiac Sarcoidosis. Ann Am Thorac Soc. 2019 Nov;16(11):1341-50. doi: 10.1513/AnnalsATS.201902-119CME.
30) 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 Mar 20;29(155):190146. doi: 10.1183/16000617.0146-2019.
31) Ballul T, Borie R, Crestani B, Daugas E, Descamps V, Dieude P, et al. Treatment of cardiac sarcoidosis: A comparative study of steroids and steroids plus immunosuppressive drugs. Int J Cardiol. 2019 Feb 1;276:208-11. doi: 10.1016/j.ijcard.2018.11.131.
32) Fussner LA, Karlstedt E, Hodge DO, Fine NM, Kalra S, Carmona EM, et al. Management and outcomes of cardiac sarcoidosis: a 20-year experience in two tertiary care centres. Eur J Heart Fail. 2018 Dec;20(12):1713-20. doi: 10.1002/ejhf.1319.
33) Nagai T, Nagano N, Sugano Y, Asaumi Y, Aiba T, Kanzaki H, et al. Effect of Corticosteroid Therapy on Long-Term Clinical Outcome and Left Ventricular Function in Patients With Cardiac Sarcoidosis. Circ J. 2015;79(7):1593-600. doi: 10.1253/circj.CJ-14-1275.
34) Harper LJ, McCarthy M, Ribeiro Neto ML, Hachamovitch R, Pearson K, Bonanno B, et al. Infliximab for Refractory Cardiac Sarcoidosis. Am J Cardiol. 2019 Nov 15;124(10):1630-5. doi: 10.1016/j.amjcard.2019.07.067.
35) Joubert B, Chapelon-Abric C, Biard L, Saadoun D, Demeret S, Dormont D, et al. Association of Prognostic Factors and Immunosuppressive Treatment With Long-term Outcomes in Neurosarcoidosis. JAMA Neurol. 2017 Nov 1;74(11):1336-44. doi: 10.1001/jamaneurol.2017.2492.
36) Marić N, Golubović A, Belić S, Đurđević N, Milivojević I, Geratović M, et al. Novi terapijski agensi u lečenju neurosarkoidoze. SMJ. Dec 2022. Vol 3, No 4 p 471-7. doi: 10.5937/smclk3-41156
37) Jovanović D, Grujičić D, Stjepanović M, Popević S, Kontić M, Vučinić Mihailović V. Unusual clinical course of neurosarcoidosis manifested with acute hydrocephalus. Acta Clin Croat. 2021 Mar;60(1):131-5. doi: 10.20471/acc.2021.60.01.19.
38) Lower EE, Broderick JP, Brott TG, et al. Diagnosis and management of neurologic sarcoidosis. Arch Intern Med 1997; 157: 1864–8. PMID: 9290546
39) Tavee JO, Karwa K, Ahmed Z, Thompson N, Parambil J, Culver DA. Sarcoidosis-associated small fiber neuropathy in a large cohort: Clinical aspects and response to IVIG and anti-TNF alpha treatment. Respir Med. 2017 May;126:135-8. doi: 10.1016/j.rmed.2017.03.011.
40) Voortman M, Fritz D, Vogels OJM, Eftimov F, van de Beek D, Brouwer MC, et al. Small fiber neuropathy: a disabling and underrecognized syndrome. Curr Opin Pulm Med. 2017 Sep;23(5):447-57. doi: 10.1097/MCP.0000000000000413.
41) Gvozdenovic BS, Mihailovic-Vucinic VV, Vukovic M, Stjepanovic MI, Buha I, Mihailovic SV, et al. Predictors of cough-specific and generic quality of life in sarcoidosis patients. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):158-68. doi: 10.36141/svdld.v37i2.9234.
42) Voortman M, Hendriks CMR, Elfferich MDP, Bonella F, Møller J, De Vries J, et al. The Burden of Sarcoidosis Symptoms from a Patient Perspective. Lung. 2019 Apr;197(2):155-61. doi: 10.1007/s00408-019-00206-7.
43) Lower EE, Malhotra A, Surdulescu V, Baughman RP. Armodafinil for sarcoidosis-associated fatigue: a double-blind, placebo-controlled, crossover trial. J Pain Symptom Manage. 2013 Feb;45(2):159-69. doi: 10.1016/j.jpainsymman.2012.02.016.
44) Peterson K, McDonagh MS, Fu R. Comparative benefits and harms of competing medications for adults with attention-deficit hyperactivity disorder: a systematic review and indirect comparison meta-analysis. Psychopharmacology (Berl). 2008 Mar;197(1):1-11. doi: 10.1007/s00213-007-0996-4.
