D. Geraint James Lecture: The sarcoidosis saga: what insights from the past will guide us in the future

Main Article Content

Robert P. Baughman
Elyse Lower

Keywords

diagnosis, therapy, centers of excellence, placebo, clinical trials

Abstract

The sarcoidosis community in general and the World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) in particular have led efforts to improve sarcoidosis diagnosis and care. Evidence based guidelines regarding the diagnosis and treatment have recently been published. In addition, several clinical trials examining existing and new treatments for sarcoidosis have been completed and published. In addition, WASOG has developed criteria and identified Centers of Excellence for sarcoidosis care around the world. In discussing what insights from the past will guide us in the future, this paper focuses on three specific topics: updating the diagnosis of sarcoidosis, using placebo-controlled trials to illuminate the natural course of pulmonary sarcoidosis; and exploring multidisciplinary sarcoidosis clinic care using Centers of Excellence. New approaches for diagnosis of sarcoidosis and steroid tapering are proposed based on current literature.

Downloads

Download data is not yet available.
Abstract 372 | PDF Downloads 284

References

(1) Crouser ED, Maier LA, Wilson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med 2020 April 15;201(8):e26-e51.
(2) Hunninghake GW, Costabel U, Ando M, et al. ATS/ERS/WASOG statement on sarcoidosis. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and other Granulomatous Disorders. Sarcoidosis Vasc Diffuse Lung Dis 1999;16(Sep):149-73.
(3) Judson MA, Baughman RP, Teirstein AS, Terrin ML, Yeager HJr, the ACCESS Research group. Defining organ involvement in sarcoidosis: the ACCESS proposed instrument. Sarcoidosis Vasc Diffuse Lung Dis 1999;16:75-86.
(4) Judson MA, Costabel U, Drent M, et al. The WASOG Sarcoidosis Organ Assessment Instrument: An update of a previous clinical tool. Sarcoidosis Vasc Diffuse Lung Dis 2014 April 18;31(1):19-27.
(5) Bickett AN, Lower EE, Baughman RP. Sarcoidosis Diagnostic Score: A Systematic Evaluation to Enhance the Diagnosis of Sarcoidosis. Chest 2018 November;154(5):1052-60.
(6) Jeny F, Vucinic V, Zhou Y, et al. Validation of the Sarcoidosis Diagnostic Score in a Multicontinental Study. Ann Am Thorac Soc 2023 March;20(3):371-80.
(7) Guleria R, Mahashur A, Ghoshal AG, Thomas PK, Raghu G, Baughman RP. Challenges in diagnosing Sarcoidosis in tuberculosis endemic regions: Clinical scenario in India. Sarcoidosis Vasc Diffuse Lung Dis 2016 December 23;33(4):381-4.
(8) Zhou Y, LI HP, Zheng H, Zhang RX, Chen G, Baughman RP. Differentiation of sarcoidosis from tuberculosis using real-time PCR assay for the detection and quantification of Mycobacterium tuberculosis. Sarcoidosis Vasc Diffuse Lung Dis 2008;25:93-9.
(9) Baughman RP, Dohn MN, Loudon RG, Frame PT. Bronchoscopy with bronchoalveolar lavage in tuberculosis and fungal infections. Chest 1991;99(1):92-7.
(10) Baughman RP, Valeyre D, Korsten P, et al. ERS clinical practice guidelines on treatment of sarcoidosis. Eur Respir J 2021 December 16;58(6):2004079-2020.
(11) Gibson GJ, Prescott RJ, Muers MF, Middleton WG, Mitchell DN, Connolly CK, Harrison BD. British Thoracic Society Sarcoidosis study: effects of long term corticosteroid treatment. Thorax 1996;51(3):238-47.
(12) Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O, the Finnish Pulmonary Sarcoidosis Study Group. Early treatment of stage II sarcoidosis improves 5-year pulmonary function. Chest 2002;121:24-31.
(13) Gottlieb JE, Israel HL, Steiner RM, Triolo J, Patrick H. Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy. Chest 1997;111(3):623-31.
(14) Baughman RP, Judson MA, Teirstein A, Yeager H, Rossman M, Knatterud GL, Thompson B. Presenting characteristics as predictors of duration of treatment in sarcoidosis. QJM 2006 May;99(5):307-15.
(15) Rizzato G, Montemurro L, Colombo P. The late follow-up of chronic sarcoid patients previously treated with corticosteroids. Sarcoidosis 1998;15:52-8.
(16) Khan NA, Donatelli CV, Tonelli AR, Wiesen J, Ribeiro Neto ML, Sahoo D, Culver DA. Toxicity risk from glucocorticoids in sarcoidosis patients. Respir Med 2017 November;132:9-14. doi: 10.1016/j.rmed.2017.09.003. Epub;%2017 Sep 8.:9-14.
(17) Rahaghi FF, Baughman RP, Saketkoo LA, et al. Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis. Eur Respir Rev 2020 March;%20;29(155):190146-2019.
(18) 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;17:60-6.
(19) Culver DA, Aryal S, Barney J, Hsia CCW, James WE, Maier LA, Marts LT, Obi ON, Sporn PHS, Sweiss NJ, Shukla S, Kinnersley N, Walker G, Baughman R. Efzofitimod for the treatment of pulmonary sarcoidosis. Chest 2022 November 7;(22):10.
(20) Crouser ED, Smith RM, Culver DA, Julian MW, Martin K, Baran J, Diaz C, Erdal BS, Hade EM. A Pilot Randomized Trial of Transdermal Nicotine for Pulmonary Sarcoidosis. Chest 2021 October;160(4):1340-9.
(21) Mirsaeidi M, Baughman RP, Sahoo D, Tarau E. Results From a Phase 4, Multicenter, Randomized, Double-Blind, Placebo-Controlled Study of Repository Corticotropin Injection for the Treatment of Pulmonary Sarcoidosis. Pulm Ther 2023 April;%19:1-17. doi: 10.1007/s41030-023-00222-2.:1-17.
(22) Drake WP, Culver DA, Baughman RP, et al. Phase II Investigation of the Efficacy of Antimycobacterial Therapy in Chronic Pulmonary Sarcoidosis. Chest 2021 May;159(5):1902-12.
(23) Baughman RP, Drent M, Kavuru M, et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med 2006 October 1;174(7):795-802.
(24) Baughman RP, Iannuzzi MC, Lower EE, et al. Use of fluticasone in acute symptomatic pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2002 October;19(3):198-204.
(25) Judson MA, Baughman RP, Costabel U, et al. Safety and efficacy of ustekinumab or golimumab in patients with chronic sarcoidosis. Eur Respir J 2014 July 17;44:1296-307.
(26) Park MK, Fontana JR, Babaali H, et al. Steroid sparing effects of pentoxifylline in pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2009;26:121-31.
(27) Baughman RP, Drent M, Culver DA, et al. Endpoints for clinical trials of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2012;29:90-8.
(28) Obi ON. Health-Related Quality of Life in Sarcoidosis. Semin Respir Crit Care Med 2020 October;41(5):716-32.
(29) Harman NL, Gorst SL, Williamson PR, et al. Scout - sarcoidosis outcomes taskforce. A systematic review of outcomes to inform the development of a core outcome set for pulmonary sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2021;38(3):e2021034.
(30) Lo KH, Donohue J, Judson MA, Wu Y, Barnathan ES, Baughman RP. The St. George's Respiratory Questionnaire in Pulmonary Sarcoidosis. Lung 2020 December;198(6):917-24.
(31) Baughman RP, Judson MA, Culver DA, Birring SS, Parambil J, Zeigler J, Lower EE. Roflumilast (Daliresp®) to reduce acute pulmonary events in fibrotic sarcoidosis: a multi-center, double blind, placebo controlled, randomized clinical trial. Sarcoidosis Vasc Diffuse Lung Dis 2021;38(3):e2021035.
(32) Baughman RP, Lower EE. Frequency of acute worsening events in fibrotic pulmonary sarcoidosis patients. Respir Med 2013 October 22;107:2009-13.
(33) Kampstra NA, van der Nat PB, Dijksman LM, et al. Results of the standard set for pulmonary sarcoidosis: feasibility and multicentre outcomes. ERJ Open Res 2019 October 30;5(4):00094-2019.
(34) Lower EE, Baughman RP. The use of low dose methotrexate in refractory sarcoidosis. Am J Med Sci 1990;299:153-7.
(35) Lower EE, Baughman RP. Prolonged use of methotrexate for sarcoidosis. Arch Intern Med 1995;155:846-51.
(36) Baughman RP, Lower EE. A clinical approach to the use of methotrexate for sarcoidosis. Thorax 1999;54:742-6.
(37) Baughman RP, Lower EE. Leflunomide for chronic sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2004;21:43-8.
(38) Baughman RP, Judson MA, Teirstein AS, Moller DR, Lower EE. Thalidomide for chronic sarcoidosis. Chest 2002;122:227-32.
(39) Baughman RP, Lower EE. Infliximab for refractory sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2001;18:70-4.
(40) Baughman RP, Bradley DA, Lower EE. Infliximab for chronic ocular inflammation. Int J Clin Pharmacol Ther 2005;43:7-11.
(41) Lower EE, Sturdivant M, Grate L, Baughman RP. Use of third-line therapies in advanced sarcoidosis. Clin Exp Rheumatol 2020 September;38(5):834-40.
(42) Baughman RP, Lower EE. Alternatives to corticosteroids in the treatment of sarcoidosis. Sarcoidosis 1997;14:121-30.
(43) Lower EE, Smith JT, Martelo OJ, Baughman RP. The anemia of sarcoidosis. Sarcoidosis 1988;5:51-5.
(44) Lower EE, Hawkins HH, Baughman RP. Breast disease in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2001 October;18(3):301-6.
(45) Lower EE, Harman S, Baughman RP. Double-blind, randomized trial of dexmethylphenidate hydrochloride for the treatment of sarcoidosis-associated fatigue. Chest 2008 May;133(5):1189-95.
(46) Lower EE, Malhotra A, Surdulescu V, Baughman RP. Armodafinil for Sarcoidosis-Associated Fatigue: A Double-Blind, Placebo-Controlled, Crossover Trial. J Pain Symptom Manage 2012 August;%20.
(47) Lower EE, Sturdivant M, Baughman RP. Presence of onconeural antibodies in sarcoidosis patients with parasarcoidosis syndrome. Sarcoidosis Vasc Diffuse Lung Dis 2019;36(4):254-60.
(48) Baughman RP, Lower EE, Bradley DA, Raymond LA, Kaufman A. Etanercept for refractory ocular sarcoidosis: results of a double-blind randomized trial. Chest 2005 August;128(2):1062-7.
(49) Lower EE, Baughman RP, Kaufman AH. Rituximab for refractory granulomatous eye disease. Clin Ophthalmol 2012;6:1613-8.
(50) Baughman RP, Lower EE, Ingledue R, Kaufman AH. Management of ocular sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2012;29:26-33.
(51) Baughman RP, Engel PJ, Meyer CA, Barrett AB, Lower EE. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2006;23:108-16.
(52) Baughman RP, Culver DA, Cordova FC, et al. Bosentan For Sarcoidosis Associated Pulmonary Arterial Hypertension (BoSAPAH): A Double Blind, Placebo Controlled Trial. Am J Resp Crit Care Med 2012;185:A3639.
(53) Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 2010 March 26;138:1078-85.
(54) Baughman RP, Koehler A, Bejarano PA, Lower EE, Weber FL, Jr. Role of liver function tests in detecting methotrexate-induced liver damage in sarcoidosis. Arch Intern Med 2003 March 10;163(5):615-20.
(55) Zhou Y, Lower EE, LI HP, Costea A, Attari M, Baughman RP. Cardiac Sarcoidosis: The Impact of Age and Implanted Devices on Survival. Chest 2017 January;151(1):139-48.
(56) Sibilia R, Baughman R, Washko D, Lower E, Costea A. Dual Medical Therapy for Treatment of Arrhythmias in Cardiac Sarcoidosis. J Innov Card Rhythm Manag 2022 November 15;13(11):5244-54.
(57) Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018 September 1;198(5):e44-e68.
(58) Ribeiro Neto ML, Jellis CL, Joyce E, Callahan TD, Hachamovitch R, Culver DA. Update in Cardiac Sarcoidosis. Ann Am Thorac Soc 2019 November;16(11):1341-50.
(59) Kron J, Syed A, Iden T, et al. Tackling Health Care Disparities: How to Build a Sarcoidosis Center. Sarcoidosis Vasc Diffuse Lung Dis 2022;39(3):e2022024.
(60) Baughman RP, Grutters JC, Wells AU, et al. World Association for Sarcoidosis and Other Granulomatous Disease (WASOG) Centers of Excellence. Sarcoidosis Vasc Diffuse Lung Dis 2022;38(4):e2021051.

Most read articles by the same author(s)

1 2 3 > >>