Current therapies for the treatment of systemic sclerosis-related pulmonary arterial hypertension: Efficacy and safety

Expert Opinion on Drug Safety

Volumn 13, Issue 3, 2014, Pages 295-305

  Murdaca, Giuseppe ^a   Spanò, Francesca ^a   Puppo, Francesco ^a  

^a UNIVERSITY OF GENOA   (Italy)

Author keywords

Anti endothelin 1; Phosphodiesterase type 5 inhibitors; Prostanoids; Systemic sclerosis related pulmonary arterial hypertension; TNF inhibitors

Indexed keywords

AMBRISENTAN; AMINOTRANSFERASE; ANTIHYPERTENSIVE AGENT; BERAPROST; BOSENTAN; ILOPROST; MACITENTAN; PROSTACYCLIN; SILDENAFIL; SITAXSENTAN; TADALAFIL; TREPROSTINIL;

AMINOTRANSFERASE BLOOD LEVEL; DIARRHEA; DRUG EFFICACY; DRUG INDUCED HEADACHE; DRUG SAFETY; DYSPEPSIA; HUMAN; LUNG HEMODYNAMICS; MYALGIA; NEBULIZATION; PULMONARY HYPERTENSION; REVIEW; SKIN DISEASE; SYSTEMIC SCLEROSIS;

ANTIHYPERTENSIVE AGENTS; HUMANS; HYPERTENSION, PULMONARY; SCLERODERMA, SYSTEMIC;

EID: 84896735712     PISSN: 14740338     EISSN: 1744764X     Source Type: Journal    
DOI: 10.1517/14740338.2014.872238     Document Type: Review

References (94)

1. Thompson AE, Pope JE. Increased prevalence of scleroderma in southwestern Ontario: A cluster analysis. J Rheumatol 2002;29:1867-73
2. Gazi H, Pope JE, Clements P, et al. Outcome measurements in scleroderma: Results from a Delphi exercise. J Rheumatol 2007;34:501-9
3. Denton CP, Hachulla E. Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening. Eur Respir Rev 2011;20(122):270-64
4. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann rheum Dis 2007;66(7):940-4
5. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, et al. Mortality in systemic sclerosis: An international meta-analysis of individual patient data. Am J Med 2005;118(1):2-10
6. Denton CP, Black CM. Pulmonary hypertension in systemic sclerosis. Rheum Dis Clin North Am 2003;29(2):335-49; vii
7. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: A French nationwide prospective multicenter study. Arthritis Rheum 2005;52(12):3792-800
8. Wigley FM, Lima JA, Mayes M, et al. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the UNCOVER study). Arthritis Rheum 2005;52(7):2125-32
9. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(12 Suppl S):13S-24S
10. Murdaca G, Colombo BM, Cagnati P, et al. Endothelial dysfunction in rheumatic autoimmune diseases. Atherosclerosis 2012;224(2):309-17
11. Murdaca G, Spanò F, Cagnati P, Puppo F. Free radicals and endothelial dysfunction: Potential positive effects of TNF-alpha inhibitors. Redox Rep 2013;18(3):95-9
12. Gonzalez MA, Selwyn AP. Endothelial function, inflammation, and prognosis in cardiovascular disease. Am J Med 2003;115(Suppl 8A):99S-106S
13. Braun-Moscovici Y, Nahir AM, Balbir-Gurman A. Endothelin and pulmonary arterial hypertension. Semin Arthritis Rheum 2004;34(1):442-53
14. Ciprandi G, Murdaca G, Colombo BM, et al. Serum vascular endothelial growth factor in allergic rhinitis and systemic lupus erythematosus. Hum Immunol 2008;69(8):510-12
15. Ciprandi G, Colombo BM, Murdaca G, De Amici M. Serum vascular endothelial growth factor and sublingual immunotherapy. Allergy 2008;63(7):945-6
16. Cool CD, Stewart JS, Werahera P, et al. Three-dimensional reconstruction of pulmonary arteries in plexiform pulmonary hypertension using cellspecific markers. Evidence for a dynamic and heterogeneous process of pulmonary endothelial cell growth. Am J Pathol 1999;155:411-19
17. El Chami H, Hassoun PM. Immune and inflammatory mechanisms in pulmonary arterial hypertension. Prog Cardiovasc Dis 2012;55(2):218-28
18. Humbert M, Monti G, Brenot F, et al. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med 1995;151:1628-31
19. Booth AD, Jayne DR, Kharbanda RK, et al. Infliximab improves endothelial dysfunction in systemic vasculitis: A model of vascular inflammation. Circulation 2004;109(14):1718-23
20. Wang Q, Zuo XR, Wang YY, et al. Monocrotaline-induced pulmonary arterial hypertension is attenuated by TNF-alpha antagonists via the suppression of TNF-alpha expression and NF-kappaB pathway in rats. Vascul Pharmacol 2013;58(1-2):71-7
21. Sutendra G, Dromparis P, Bonnet S, et al. Pyruvate dehydrogenase inhibition by the inflammatory cytokine TNF-alpha contributes to the pathogenesis of pulmonary arterial hypertension. J Mol Med (Berl) 2011;89(8):771-83
22. Truchetet ME, Brembilla NC, Montanari E, et al. Interleukin-17A+ cell counts are increased in systemic sclerosis skin and their number is inversely correlated with the extent of skin involvement. Arthritis Rheum 2013;65(5):1347-56
23. Murdaca G, Colombo BM, Puppo F. The role of Th17 lymphocytes in the autoimmune and chronic inflammatory diseases. Intern Emerg Med 2011;6(6):487-95
24. Colombo BM, Cacciapaglia F, Puntoni M, et al. Traditional and non traditional risk factors in accelerated atherosclerosis in systemic lupus erythematosus: Role of vascular endothelial growth factor (VEGATS Study). Autoimmun Rev 2009;8(4):309-15
25. Colombo BM, Murdaca G, Caiti M, et al. Intima-media thickness: A marker of accelerated atherosclerosis in women with systemic lupus erythematosus. Ann NY Acad Sci 2007;1108:121-6
26. Kawut SM, Taichman DB, Archer-Chicko CL, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003;123(2):344-50
27. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010;122:156-63
28. Thenappan T, Shah SJ, Rich S, et al. Survival in pulmonary arterial hypertension: A reappraisal of the NIH risk stratification equation. Eur Respir J 2010;35:1079-87
29. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: A study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 2010;69:1809-15
30. Humbert M, Sitbon O, Yai?ci A, et al. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010;36:549-55
31. Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009;179:151-7
32. Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010;121:20-5
33. Kenyon KW, Nappi JM. Bosentan for the treatment of pulmonary arterial hypertension. Ann Pharmacother 2003;37(7-8):1055-62
34. Kowal-Bielecka O, Landewé R, Avouac J, et al. EUSTAR Co-Authors. EULAR recommendations for the treatment of systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009;68(5):620-8
35. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346(12):896-903
36. Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc 1982;14(5):377-81
37. Humbert M, Barst RJ, Robbins IM, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004;24(3):353-9
38. Sasayama S, Kunieda T, Tomoike H, et al. Effects of the endothelin receptor antagonist bosentan on hemodynamics, symptoms and functional capacity in Japanese patients with severe pulmonary hypertension. Circ J 2005;69(2):131-7
39. Dimitroulas T, Giannakoulas G, Karvounis H, et al. N-terminal pro-brain natriuretic peptide in systemic sclerosis related pulmonary arterial hypertension under bosentan treatment. Rheumatol Int 2009;29(3):347-8
40. Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebocontrolled study. Circulation 2006;114:48-54
41. McGoon MD, Frost AE, Oudiz RJ, et al. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest 2009;135:122-9
42. Galiè N, Olschewski H, Oudiz RJ, et al. Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double- Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. Ambrisentan for the treatment of pulmonary arterial hypertension: Results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008;117(23):3010-19
43. Oudiz RJ, Galiè N, Olschewski H, et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(21):1971-81
44. Badesch DB, Feldman J, Keogh A, et al. ARIES-3: Ambrisentan therapy in a diverse population of patients with pulmonary hypertension. Cardiovasc Ther 2012;30(2):93-9
45. Elshaboury SM, Anderson Jr. Ambrisentan for the treatment of pulmonary arterial hypertension: Improving outcomes. Patient Prefer Adherence 2013;7:401-9
46. Vizza CD, Fedele F, Pezzuto B, Rubin LJ. Safety and efficacy evaluation of ambrisentan in pulmonary hypertension. Expert Opin Drug Saf 2012;11(6):1003-11
47. Maron BA, Waxman AB, Opotowsky AR, et al. Effectiveness of spironolactone plus ambrisentan for treatment of pulmonary arterial hypertension (from the [ARIES] study 1 and 2 trials). Am J Cardiol 2013;112(5):720-5
48. Iglarz M, Binkert C, Morrison K, et al. Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J Pharmacol Exp Ther 2008;327(3):736-45
49. Gatfield J, Mueller Grandjean C, Sasse T, et al. Slow receptor dissociation kinetics differentiate macitentan from other endothelin receptor antagonists in pulmonary arterial smooth muscle cells. PLoS One 2012;7(10):e47662
50. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369(9):809-18
51. Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169(4):441-7
52. Lavelle A, Sugrue R, Lawler G, et al. Sitaxentan-induced hepatic failure in two patients with pulmonary arterial hypertension. Eur Respir J 2009;34(3):770-1
53. Klinger Jr. The nitric oxide/cGMP signaling pathway in pulmonary hypertension. Clin Chest Med 2007;28(1):143-67
54. Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353(20):2148-57
55. Rubin LJ, Badesch DB, Fleming TR, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: The SUPER-2 study. Chest 2011;140(5):1274-83
56. Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119(22):2894-903
57. Oudiz RJ, Brundage BH, Galiè N, et al. Tadalafil for the treatment of pulmonary arterial hypertension: A double-blind 52- week uncontrolled extension study. J Am Coll Cardiol 2012;60(8):768-74
58. Shapiro S, Traiger G, Hill W, et al. Safety, tolerability, and efficacy of overnight switching from sildenafil to tadalafil in patients with pulmonary arterial hypertension. Cardiovasc Ther 2013;31(5):274-9
59. Aggarwal P, Patial RK, Negi PC, Marwaha R. Oral tadalafil in pulmonary artery hypertension: A prospective study. Indian Heart J 2007;59(4):329-35
60. Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol 2012;33(5):683-8
61. Maki H, Yao A, Inaba T, et al. Initial and programmed combination therapy with oral drugs for severe idiopathic pulmonary arterial hypertension. Int Heart J 2011;52(5):323-6
62. Liang F, Yang S, Yao L, et al. Ambrisentan and tadalafil synergistically relax endothelin-induced contraction of rat pulmonary arteries. Hypertension 2012;59(3):705-11
63. Sidorenko BA, Preobrazhenskii DV, Batyraliev TA, Belenkov IuN. Pulmonary arterial hypertension: Changing approaches to management. Kardiologiia 2011;51(1):100-8
64. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002;347(5):322-9
65. Opitz CF, Wensel R, Winkler J, et al. Clinical efficacy and survival with firstline inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J 2005;26(18):1895-902
66. Lopez-Meseguer M, Berastegui C, Monforte V, et al. Inhaled iloprost plus oral sildenafil in patients with severe pulmonary arterial hypertension delays the need for lung transplantation. Transplant Proc 2013;45(6):2347-50
67. Barst RJ, McGoon M, McLaughlin V, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003;41(12):2119-25
68. Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990;112(7):485-91
69. Barst RJ, Rubin LJ, McGoon MD, et al. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med 1994;121(6):409-15
70. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334(5):296-301
71. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: A double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002;165(6):800-4
72. McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: A randomized controlled clinical trial. J Am Coll Cardiol 2010;55(18):1915-22
73. McLaughlin VV, Gaine SP, Barst RJ, et al. Efficacy and safety of treprostinil: An epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol 2003;41(2):293-9
74. Jing ZC, Parikh K, Pulido T, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: A randomized, controlled trial. Circulation 2013;127(5):624-33
75. Hoeper MM, Barst RJ, Bourge RC, et al. Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: Results of the randomized IMPRES study. Circulation 2013;127(10):1128-38
76. Murdaca G, Colombo BM, Cagnati P, et al. Update upon efficacy and safety of TNF-alpha inhibitors. Expert Opin Drug Saf 2012;11(1):1-5
77. Murdaca G, Colombo BM, Puppo F. Emerging biological drugs: A new therapeutic approach for Systemic Lupus Erythematosus. An update upon efficacy and adverse events. Autoimmun Rev 2011;11(1):56-60
78. Murdaca G, Colombo BM, Puppo F. Adalimumab for the treatment of immune-mediated diseases: An update on old and recent indications. Drugs Today (Barc) 2011;47(4):277-88
79. Murdaca G, Colombo BM, Puppo F. Anti-TNF-alpha inhibitors: A new therapeutic approach for inflammatory immune-mediated diseases: An update upon efficacy and adverse events. Int J Immunopathol Pharmacol 2009;22(3):557-65
80. Murdaca G, Colombo BM, Barabino G, et al. Anti-tumor necrosis factor-alpha treatment with infliximab for disseminated granuloma annulare. Am J Clin Dermatol 2010;11(6):437-9
81. Puppo F, Murdaca G, Ghio M, Indiveri F. Emerging biologic drugs for the treatment of rheumatoid arthritis. Autoimmun Rev 2005;4(8):537-41
82. Murdaca G, Spanò F, Puppo F. Use of leflunomide plus TNF-alpha inhibitors in rheumatoid arthritis. Expert Opin Drug Saf 2013;12(6):801-4
83. Murdaca G, Spanò F, Puppo F. Selective TNF-alpha inhibitor-induced injection site reactions. Expert Opin Drug Saf 2013;12(2):187-93
84. Murdaca G, Colombo BM, Contini P, Puppo F. Determination of lymphotoxin-alpha levels in patients with psoriatic arthritis undergoing etanercept treatment. J Interferon Cytokine Res 2012;32(6):277-9
85. Omair MA, Phumethum V, Johnson SR. Long-term safety and effectiveness of tumour necrosis factor inhibitors in systemic sclerosis patients with inflammatory arthritis. Clin Exp Rheumatol 2012;30(2 Suppl 71):S55-9
86. Arenas IA, Armstrong SJ, Xu Y, Davidge ST. Chronic tumor necrosis factor-alpha inhibition enhances NO modulation of vascular function in estrogen-deficient rats. Hypertension 2005;46(1):76-81
87. Dulai R, Perry M, Twycross-Lewis R, et al. The effect of tumor necrosis factora antagonists on arterial stiffness in rheumatoid arthritis: A literature review. Semin Arthritis Rheum 2012;42(1):1-8
88. Szekanecz Z, Kerekes G, Soltész P. Vascular effects of biologic agents in RA and spondyloarthropathies. Nat Rev Rheumatol 2009;5(12):677-84
89. Murdaca G, Spanò F, Miglino M, Puppo F. Effects of TNF-a inhibitors upon the mechanisms of action of VEGF. Immunotherapy 2013;5(2):113-15
90. Bargagli E, Galeazzi M, Bellisai F, et al. Infliximab treatment in a patient with systemic sclerosis associated with lung fibrosis and pulmonary hypertension. Respiration 2008;75:346-9
91. Wang Q, Zuo XR, Wang YY, et al. Monocrotaline-induced pulmonary arterial hypertension is attenuated by TNF-alpha antagonists via the suppression of TNF-alpha expression and NF-kappaB pathway in rats. Vascul Pharmacol 2013;58(1-2):71-7
92. Hochberg MC, Lebwohl MG, Plevy SE, et al. The benefit/risk profile of TNFblocking agents: Findings of a consensus panel. Semin Arthritis Rheum 2005;34:819-36
93. Rosenblum H, Amital H. Anti-TNF therapy: Safety aspects of taking the risk. Autoimmun Rev 2011;10:563-8
94. Bender NK, Heilig CE, Dr€oll B, et al. Immunogenicity, efficacy and adverse events of adalimumab in RA patients. Rheumatol Int 2007;27:269-74