Therapy for sarcoidosis: Evidence-based recommendations

Expert Review of Clinical Immunology

Volumn 8, Issue 1, 2012, Pages 95-103

  Baughman, Robert P ^a   Nunes, Hilario ^b  

^a UNIVERSITY OF CINCINNATI COLLEGE OF MEDICINE   (United States)

^b AP HP   (France)

Author keywords

azathioprine; infliximab; methotrexate; prednisone; sarcoidosis

Indexed keywords

AZATHIOPRINE; CORTICOSTEROID; CYCLOSPORIN; FLUTICASONE; INFLIXIMAB; METHOTREXATE; PENTOXIFYLLINE; PREDNISONE; THALIDOMIDE;

CORTICOSTEROID THERAPY; DRUG CHOICE; EVIDENCE BASED MEDICINE; HUMAN; LONG TERM CARE; REVIEW; SARCOIDOSIS; SYSTEMIC THERAPY;

EVIDENCE-BASED MEDICINE; HUMANS; PRACTICE GUIDELINES AS TOPIC; SARCOIDOSIS;

EID: 83455244339     PISSN: 1744666X     EISSN: 17448409     Source Type: Journal    
DOI: 10.1586/eci.11.84     Document Type: Review

References (105)

1. 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. 16, 149-173 (1999).
2. Baughman RP, Teirstein AS, Judson MA et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am. J. Respir. Crit. Care Med. 164, 1885-1889 (2001).
3. Rizzato G, Montemurro L, Colombo P. The late follow-up of chronic sarcoid patients previously treated with corticosteroids. Sarcoidosis 15, 52-58 (1998).
4. Gottlieb JE, Israel HL, Steiner RM, Triolo J, Patrick H. Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy. Chest 111(3), 623-631 (1997).
5. Gibson GJ, Prescott RJ, Muers MF et al. British thoracic society sarcoidosis study: effects of long term corticosteroid treatment. Thorax 51(3), 238-247 (1996).
6. Baughman RP, Judson MA, Teirstein A et al. Presenting characteristics as predictors of duration of treatment in sarcoidosis. Q. J. Med. 99(5), 307-315 (2006).
7. Baughman RP, Costabel U, du Bois RM. Treatment of sarcoidosis. Clin. Chest Med. 29(3), 533-548 (2008).
8. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q. J. Med. 208, 525-533 (1983).
9. Grunewald J, Eklund A. Lofgrens syndrome: human leukocyte antigen strongly influences the disease course. Am. J. Respir. Crit. Care Med. 179(4), 307-312 (2009).
10. Bogunia-Kubik K, Tomeczko J, Suchnicki K, Lange A. HLA-DRB1*03, DRB1*11 or DRB1*12 and their respective DRB3 specificities in clinical variants of sarcoidosis. Tissue Antigens 57(1), 87-90 (2001).
11. Wijnen PA, Nelemans PJ, Verschakelen JA, Bekers O, Voorter CE, Drent M. The role of tumor necrosis factor a G-308A polymorphisms in the course of pulmonary sarcoidosis. Tissue Antigens 75(3), 262-268 (2010).
12. Grubic Z, Peros-Golubicic T, Stingl K, Zunec R. The investigation of HLA microsatellites influence in predisposition to sarcoidosis among Croatians. Sarcoidosis Vasc. Diffuse Lung Dis. 28(1), 18-26 (2011).
13. Johns CJ, Michele TM. The clinical management of sarcoidosis: a 50-year experience at the Johns Hopkins hospital. Medicine 78, 65-111 (1999).
14. Baughman RP, Lower EE, Tami T. Upper airway. 4: sarcoidosis of the upper respiratory tract (SURT). Thorax 65(2), 181-186 (2010).
15. Baughman RP, Nagai S, Balter M et al. Defining the clinical outcome status (COS) in sarcoidosis: results of WASOG task force. Sarcoidosis Vasc. Diffuse Lung Dis. 28(1), 56-64 (2011).
16. Stagaki E, Mountford WK, Lackland DT, Judson MA. The treatment of lupus pernio: results of 116 treatment courses in patients. Chest 135(2), 468-476 (2009).
17. Lower EE, Broderick JP, Brott TG, Baughman RP. Diagnosis and management of neurologic sarcoidosis. Arch. Intern. Med. 157, 1864-1868 (1997).
18. Chambellan A, Turbie P, Nunes H, Brauner M, Battesti JP, Valeyre D. Endoluminal stenosis of proximal bronchi in sarcoidosis: bronchoscopy, function, and evolution. Chest 127(2), 472-481 (2005).
19. Baughman RP, Selroos O. Evidence-based approach to the treatment of sarcoidosis. In:Evidence-Based Respiratory Medicine . Gibson PG, Abramson M, Wood-Baker R, Volmick J, Hensley M, Costabel U (Eds). Malden, Blackwell Publishing Ltd., Oxford, UK, 491-508 (2005).
20. Paramothayan S, Jones PW. Corticosteroid therapy in pulmonary sarcoidosis: a systematic review. JAMA 287, 1301-1307 (2002).
21. Paramothayan S, Lasserson T, Walters EH. Immunosuppressive and cytotoxic therapy for pulmonary sarcoidosis. Cochrane Database Syst. Rev. 3, CD003536 (2003).
22. Bradley B, Branley HM, Egan JJ 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 63(Suppl. 5), S1-S58 (2008).
23. Scadding JG. Prognosis of intrathoracic sarcoidosis in England. Br. Med. J. 4, 1165-1172 (1961).
24. Loddenkemper R, Kloppenborg A, Schoenfeld N, Grosser H, Costabel U. Clinical findings in 715 patients with newly detected pulmonary sarcoidosis-results of a cooperative study in former West Germany and Switzerland. WATL study group. Wissenschaftliche arbeitsgemeinschaft fur die therapie von lungenkrankheitan. Sarcoidosis Vasc. Diffuse Lung Dis. 15(2), 178-182 (1998).
25. Nagai S, Shigematsu M, Hamada K, Izumi T. Clinical courses and prognoses of pulmonary sarcoidosis. Curr. Opin. Pulm. Med. 5(5), 293-298 (1999).
26. Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O. Finnish pulmonary sarcoidosis study group. Oral prednisolone followed by inhaled budesonide in newly diagnosed pulmonary sarcoidosis: a double-blind, placebo-controlled, multicenter study. Chest 116, 424-431 (1999).
27. Yeager H, Rossman MD, Baughman RP et al. Pulmonary and psychosocial findings at enrollment in the ACCESS study. Sarcoidosis Vasc. Diffuse Lung Dis. 22(2), 147-153 (2005).
28. Baughman RP, Shipley R, Desai S et al. Changes in chest roentgenogram of sarcoidosis patients during a clinical trial of infliximab therapy: comparison of different methods of evaluation. Chest 136, 526-535 (2009).
29. Oberstein A, von Zitzewitz H, Schweden F, Muller-Quernheim J. Non invasive evaluation of the inflammatory activity in sarcoidosis with high-resolution computed tomography. Sarcoidosis Vasc. Diffuse Lung Dis. 14(1), 65-72 (1997).
30. Sulica R, Teirstein AS, Kakarla S, Nemani N, Behnegar A, Padilla ML. Distinctive clinical, radiographic, and functional characteristics of patients with sarcoidosis-related pulmonary hypertension. Chest 128(3), 1483-1489 (2005).
31. Baughman RP, Engel PJ, Taylor L, Lower EE. Survival in sarcoidosis associated pulmonary hypertension: the importance of hemodynamic evaluation. Chest 138, 1078-1085 (2010).
32. Nunes H, Humbert M, Capron F et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61(1), 68-74 (2006).
33. Spruit MA, Thomeer MJ, Gosselink R et al. Skeletal muscle weakness in patients with sarcoidosis and its relationship with exercise intolerance and reduced health status. Thorax 60(1), 32-38 (2005).
34. Baydur A, Alsalek M, Louie SG, Sharma OP. Respiratory muscle strength, lung function, and dyspnea in patients with sarcoidosis. Chest 120(1), 102-108 (2001).
35. Robinson LR, Brownsberger R, Raghu G. Respiratory failure and hypoventilation secondary to neurosarcoidosis. Am. J. Respir. Crit. Care Med. 157(4 Pt 1), 1316-1318 (1998).
36. de Kleijn WP, de Vries J, Lower EE, Elfferich MD, Baughman RP, Drent M. Fatigue in sarcoidosis: a systematic review. Curr. Opin. Pulm. Med. 15(5), 499-506 (2009).
37. Baughman RP, Sparkman BK, Lower EE. Six-minute walk test and health status assessment in sarcoidosis. Chest 132(1), 207-213 (2007).
38. Pietinalho A, Lindholm A, Haahtela T, Tukiainen P, Selroos O. Inhaled budesonide for treatment of pulmonary sarcoidosis. Results of a double-blind, placebo-controlled, multicentre study. Eur. Respir. J. 9(2 Suppl. 23), S406 (1996).
39. Pietinalho A, Tukiainen P, Haahtela T, Persson T, Selroos O. The Finnish Pulmonary Sarcoidosis Study Group. Early treatment of stage 2 sarcoidosis improves 5-year pulmonary function. Chest 121, 24-31 (2002).
40. McKinzie BP, Bullington WM, Mazur JE, Judson MA. Efficacy of short-course, low-dose corticosteroid therapy for acute pulmonary sarcoidosis exacerbations. Am. J. Med. Sci. 339(1), 1-4 (2010).
41. Schutt AC, Bullington WM, Judson MA. Pharmacotherapy for pulmonary sarcoidosis: a Delphi consensus study. Respir. Med. 104(5), 717-723 (2010).
42. 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. 236(3), 285-290 (1994).
43. Baughman RP, Iannuzzi MC, Lower EE et al. Use of fluticasone in acute symptomatic pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 19(3), 198-204 (2002).
44. du Bois RM, Greenhalgh PM, Southcott AM, Johnson NM, Harris TA. Randomized trial of inhaled fluticasone propionate in chronic follow-up pulmonary sarcoidosis: a pilot study. Eur. Respir. J. 13(6), 1345-1350 (1999).
45. Lower EE, Baughman RP. Prolonged use of methotrexate for sarcoidosis. Arch. Intern. Med. 155, 846-851 (1995).
46. Vucinic VM. What is the future of methotrexate in sarcoidosis? A study and review. Curr. Opin. Pulm. Med. 8(5), 470-476 (2002).
47. 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. 17, 60-66 (2000).
48. Webster GF, Razsi LK, Sanchez M, Shupack JL. Weekly low-dose methotrexate therapy for cutaneous sarcoidosis. J. Am. Acad. Dermatol. 24, 451-454 (1991).
49. Dev S, McCallum RM, Jaffe GJ. Methotrexate for sarcoid-associated panuveitis. Ophthalmology 106, 111-118 (1999).
50. Samson CM, Waheed N, Baltatzis S, Foster CS. Methotrexate therapy for chronic nonifectious uveitis: analysis of a case series of 160 patients. Ophthalmology 108, 1134-1139 (2001).
51. Baughman RP, Lower EE, Bradley DA, Kaufman AH. Use of cytotoxic therapy for chronic opthalmic sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 16, S17 (1999).
52. Lower EE, Baughman RP. The use of low dose methotrexate in refractory sarcoidosis. Am. J. Med. Sci. 299, 153-157 (1990).
53. Baughman RP, Koehler A, Bejarano PA, Lower EE, Weber FL Jr. Role of liver function tests in detecting methotrexateinduced liver damage in sarcoidosis. Arch. Intern. Med. 163(5), 615-620 (2003).
54. Zisman DA, McCune WJ, Tino G, Lynch JP 3rd. Drug-induced pneumonitis: the role of methotrexate. Sarcoidosis Vasc. Diffuse Lung Dis. 18(3), 243-252 (2001).
55. Muller-Quernheim J, Kienast K, Held M, Pfeifer S, Costabel U. Treatment of chronic sarcoidosis with an azathioprine/ prednisolone regimen. Eur. Respir. J. 14(5), 1117-1122 (1999).
56. Baughman RP, Lower EE. Alternatives to corticosteroids in the treatment of sarcoidosis. Sarcoidosis 14, 121-130 (1997).
57. Lewis SJ, Ainslie GM, Bateman ED. Efficacy of azathioprine as second-line treatment in pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 16, 87-92 (1999).
58. Kennedy PT, Zakaria N, Modawi SB et al. Natural history of hepatic sarcoidosis and its response to treatment. Eur. J. Gastroenterol. Hepatol. 18(7), 721-726 (2006).
59. Baughman RP, Lower EE. Leflunomide for chronic sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 21, 43-48 (2004).
60. Sahoo DH, Bandyopadhyay D, Xu M et al. Effectiveness and safety of leflunomide for pulmonary and extrapulmonary sarcoidosis. Eur. Respir. J. 38(5), 1145-1150 (2011).
61. Kremer JM, Caldwell JR, Cannon GW et al. The combination of leflunomide and methotrexate in patients with active rheumatoid arthritis who are failing on methotrexate treatment alone: a doubleblind placebo controlled study. Arthritis Rheum. 43, S224 (2000).
62. Emery P, Breedveld FC, Lemmel EM et al. A comparison of the efficacy and safety of leflunomide and methotrexate for the treatment of rheumatoid arthritis. Rheumatology (Oxford) 39(6), 655-665 (2000).
63. Ziegenhagen MW, Rothe E, Zissel G, Muller-Quernheim J. Exaggerated TNFa release of alveolar macrophages in corticosteroid resistant sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 19, 185-190 (2002).
64. Baughman RP, Lower EE, Drent M. Inhibitors of tumor necrosis factor (TNF) in sarcoidosis: who, what, and how to use them. Sarcoidosis Vasc. Diffuse Lung Dis. 25, 76-89 (2008).
65. Baughman RP, Lower EE. Infliximab for refractory sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 18, 70-74 (2001).
66. Doty JD, Mazur JE, Judson MA. Treatment of sarcoidosis with infliximab. Chest 127(3), 1064-1071 (2005).
67. Saleh S, Ghodsian S, Yakimova V, Henderson J, Sharma OP. Effectiveness of infliximab in treating selected patients with sarcoidosis. Respir. Med. 100(11), 2053-2059 (2006).
68. Baughman RP, Bradley DA, Lower EE. Infliximab for chronic ocular inflammation. Int. J. Clin. Pharmacol. Ther. 43, 7-11 (2005).
69. Baughman RP, Drent M, Kavuru M et al. Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am. J. Respir. Crit. Care Med. 174(7), 795-802 (2006).
70. Rossman MD, Newman LS, Baughman RP et al. A double-blind, randomized, placebo-controlled trial of infliximab in patients with active pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 23, 201-208 (2006).
71. Judson MA, Baughman RP, Costabel U et al. Efficacy of infliximab in extrapulmonary sarcoidosis: results from a randomised trial. Eur. Respir. J. 31(6), 1189-1196 (2008).
72. Moravan M, Segal BM. Treatment of CNS sarcoidosis with infliximab and mycophenolate mofetil. Neurology 72(4), 337-340 (2009).
73. Sodhi M, Pearson K, White ES, Culver DA. Infliximab therapy rescues cyclophosphamide failure in severe central nervous system sarcoidosis. Respir. Med. 103(2), 268-273 (2009).
74. Lahmer T, Knopf A, Lanzl I, Heemann U, Thuermel K. Using TNF-a antagonist Adalimumab for treatment for multisystem sarcoidosis: a case study. Rheumatol. Int. doi:10.1007/s00296-011- 1968-x (2011) (Epub ahead of print).
75. Baughman RP. Tumor necrosis factor inhibition in treating sarcoidosis: the American experience. Revista Portuguesa de Pneumonologia 13, S47-S50 (2007).
76. Field S, Regan AO, Sheahan K, Collins P. Recalcitrant cutaneous sarcoidosis responding to adalimumab but not to etanercept. Clin. Exp. Dermatol. 35(7), 795-796 (2010).
77. Sandborn WJ, Rutgeerts P, Enns R et al. Adalimumab induction therapy for Crohn disease previously treated with infliximab: a randomized trial. Ann. Intern. Med. 19, 146(12), 829-838 (2007).
78. Sandborn WJ, Hanauer SB, Rutgeerts P et al. Adalimumab for maintenance treatment of Crohns disease: results of the CLASSIC II trial. Gut 56(9), 1232-1239 (2007).
79. Chloroquine in the treatment of sarcoidosis. A report from the Research Committee of the British Tuberculosis Association. Tubercle 48 257-272 (1967)
80. Siltzbach LE, Teirstein AS. Chloroquine therapy in 43 patients with intrathoracic and cutaneous sarcoidosis. Acta Med. Scand. 425, S302-S308 (1964).
81. Baltzan M, Mehta S, Kirkham TH, Cosio MG. Randomized trial of prolonged chloroquine therapy in advanced pulmonary sarcoidosis. Am. J. Respir. Crit. Care Med. 160(1), 192-197 (1999).
82. Kouba DJ, Mimouni D, Rencic A, Nousari HC. Mycophenolate mofetil may serve as a steroid-sparing agent for sarcoidosis. Br. J. Dermatol. 148(1), 147-148 (2003).
83. Varron L, Broussolle C, Candessanche JP et al. Spinal cord sarcoidosis: report of seven cases. Eur. J. Neurol. 16(3), 289-296 (2009).
84. Bichari W, Bartiromo M, Mohey H et al. Significant risk factors for occurrence of cancer after renal transplantation: a single center cohort study of 1265 cases. Transplant. Proc. 41(2), 672-673 (2009).
85. Marcen R. Immunosuppressive drugs in kidney transplantation: impact on patient survival, and incidence of cardiovascular disease, malignancy and infection. Drugs 69(16), 2227-2243 (2009).
86. Zabel P, Entzian P, Dalhoff K, Schlaak M. Pentoxifylline in treatment of sarcoidosis. Am. J. Respir. Crit. Care Med. 155, 1665-1669 (1997).
87. Park MK, Fontana JR, Babaali H et al. Steroid sparing effects of pentoxifylline in pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 26, 121-131 (2009).
88. Schafer PH, Parton A, Gandhi AK et al. Apremilast, a cAMP phosphodiesterase-4 inhibitor, demonstrates anti-inflammatory activity in vitro and in a model of psoriasis. Br. J. Pharmacol. 159(4), 842-855 (2010).
89. Baughman RP, Judson MA, Teirstein AS, Moller DR, Lower EE. Thalidomide for chronic sarcoidosis. Chest 122, 227-232 (2002).
90. Nguyen YT, Dupuy A, Cordoliani F et al. Treatment of cutaneous sarcoidosis with thalidomide. J. Am. Acad. Dermatol. 50(2), 235-241 (2004).
91. Belkhou A, Younsi R, El Bouchti I, El Hassani S. Rituximab as a treatment alternative in sarcoidosis. Joint Bone Spine 75(4), 511-512 (2008).
92. Dasilva V, Breuil V, Chevallier P, Euller-Ziegler L. Relapse of severe sarcoidosis with an uncommon peritoneal location after TNFα blockade. Efficacy of rituximab, report of a single case. Joint Bone Spine 77(1), 82-83 (2010).
93. Aksoy S, Harputluoglu H, Kilickap S et al. Rituximab-related viral infections in lymphoma patients. Leuk. Lymphoma. 48(7), 1307-1312 (2007).
94. Gribbin J, Hubbard RB, Le JI, Smith CJ, West J, Tata LJ. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 61(11), 980-985 (2006).
95. Swigris JJ, Olson AL, Huie TJ et al. Sarcoidosis-related mortality in the United States from 1988 to 2007. Am. J. Respir. Crit. Care Med. 183(11), 1524-1530 (2011).
96. Baughman RP, Winget DB, Bowen EH, Lower EE. Predicting respiratory failure in sarcoidosis patients. Sarcoidosis 14, 154-158 (1997).
97. Reich JM. Mortality of intrathoracic sarcoidosis in referral vs population-based settings: influence of stage, ethnicity, and corticosteroid therapy. Chest 121, 32-39 (2002).
98. Baughman RP, Nagai S, Balter M et al. Defining the clinical outcome status (COS) in sarcoidosis: results of WASOG task force. Sarcoidosis Vasc. Diffuse Lung Dis. 28(1), 56-64 (2011).
99. James DG, Carstairs LS, Trowell J, Sharma OP. Treatment of sarcoidosis: report of a controlled therapeutic trial. Lancet 2, 526-528 (1967).
100. Israel HL, Fouts DW, Beggs RA. A controlled trial of prednisone treatment of sarcoidosis. Am. Rev. Respir. Dis. 107, 609-614 (1973).
101. Selroos O, Sellergren TL. Corticosteroid therapy of pulmonary sarcoidosis. Scand. J. Respir. Dis. 60, 215-221 (1979).
102. Zaki MH, Lyons HA, Leilop L, Huang CT. Corticosteroid therapy in sarcoidosis: a five year controlled follow-up. NY State J. Med. 87, 496-499 (1987).
103. Judson MA, Silvestri J, Hartung C, Byars T, Cox CE. The effect of thalidomide on corticosteroid-dependent pulmonary sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 23(1), 51-57 (2006).
104. Wyser CP, van Schalkwyk EM, Alheit B, Bardin PG, Joubert JR. Treatment of progressive pulmonary sarcoidosis with cyclosporin A: a randomized controlled trial. Am. J. Respir. Crit. Care Med. 156, 1571-1576 (1997).
105. Sweiss NJ, Barnathan ES, Lo K, Judson MA, Baughman R. C-reactive protein predicts response to infliximab in patients with chronic sarcoidosis. Sarcoidosis Vasc. Diffuse Lung Dis. 27, 49-56 (2010).