Therapy for pulmonary arterial hypertension associated with systemic sclerosis

Current Opinion in Rheumatology

Volumn 21, Issue 6, 2009, Pages 642-648

  Mathai, Stephen C ^a   Hassoun, Paul M ^a,b  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b Division of Pulmonary and Critical Care Medicine   (United States)

Author keywords

Diagnosis; Pulmonary hypertension; Systemic sclerosis; Therapy

Indexed keywords

AMBRISENTAN; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; PHOSPHODIESTERASE INHIBITOR; PROSTACYCLIN; PROSTAGLANDIN; SILDENAFIL; SITAXSENTAN;

ANTICOAGULATION; CELL PROLIFERATION; DISEASE ASSOCIATION; DRUG EFFICACY; DRUG MEGADOSE; HUMAN; LOW DRUG DOSE; LUNG BLOOD VESSEL; LUNG TRANSPLANTATION; OUTCOME ASSESSMENT; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RAYNAUD PHENOMENON; REVIEW; SYSTEMIC SCLEROSIS;

ALGORITHMS; ANTICOAGULANTS; CALCIUM CHANNEL BLOCKERS; DRUG THERAPY, COMBINATION; EPOPROSTENOL; EVIDENCE-BASED MEDICINE; HUMANS; HYPERTENSION, PULMONARY; LUNG TRANSPLANTATION; PHOSPHODIESTERASE INHIBITORS; RECEPTORS, ENDOTHELIN; SCLERODERMA, SYSTEMIC;

EID: 70349843635     PISSN: 10408711     EISSN: None     Source Type: Journal    
DOI: 10.1097/BOR.0b013e3283307dc8     Document Type: Review

References (66)

1. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation 2009; 119:2250-2294.
2. Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003; 62:1088-1093.
3. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multi-center study. Arthritis Rheum 2005; 52:3792-3800.
4. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007; 66:940-944.
5. Kawut SM, Taichman DB, Archer-Chicko CL, et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003; 123:344-350.
6. Fisher MR, Mathai SC, Champion HC, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006; 54:3043-3050.
7. McLaughlin VV, Presberg KW, Doyle RL, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004; 126:78S-92S.
8. Tamaki T, Mori S, Takehara K. Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res 1991; 283:366-371.
9. Allcock RJ, Forrest I, Corris PA, et al. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology (Oxford) 2004; 43:596-602.
10. Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am 2003; 29:239-254.
11. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173:1023-1030.
12. Badesch DB, Benza RL, Krichman AM, et al. REVEAL Registry: Baseline characteristics of the first 1,226 enrolled patients [abstract]. Chest 2007; 132:473S.
13. Wigley FM, Lima JA, Mayes M, et al. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary healthcare level of community-based rheumatologists (the UNCOVER study). Arthritis Rheum 2005; 52:2125-2132.
14. Mangat P, Conron M, Gabbay E, Proudman S. Scleroderma lung disease: variation in screening, diagnosis and treatment practices between rheumatologists and respiratory physicians. Intern Med J 2009. [Epub ahead of print]
15. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003; 48:516-522. (Pubitemid 36278021)
16. Allanore Y, Borderie D, Avouac J, et al. High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 2008; 58:284-291.
17. Campo A, Mathai SC, Zaiman A, et al. Survival of pulmonary arterial hypertension related to systemic sclerosis: a single center longitudinal study [abstract]. Proc Am Thorac Soc 2009; A2657.
18. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension: comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000; 161:487-492.
19. Gilbert C, Brown MC, Cappelleri JC, et al. Estimating a minimally important difference in pulmonary arterial hypertension following treatment with silde nafil. Chest 2009; 135:137-142.
20. Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353:2148-2157. (Pubitemid 41653106)
21. Garin MC, Highland KB, Silver RM, Strange C. Limitations to the 6-min walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol 2009; 36:330-336.
22. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40:780-788.
23. Provencher S, Sitbon O, Humbert M, et al. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J 2006; 27:589-595.
24. Enright PL, Sherrill DL. Reference equations for the six-minute walk in healthy adults. Am J Respir Crit Care Med 1998; 158:1384-1387.
25. Nagaya N, Uematsu M, Satoh T, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999; 160:487-492.
26. Forfia PR, Mathai SC, Fisher MR, et al. Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension. Am J Respir Crit Care Med 2008; 177:1364-1369.
27. Fijalkowska A, Kurzyna M, Torbicki A, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 2006; 129:1313-1321.
28. Williams MH, Handler CE, Akram R, et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 2006; 27:1485-1494.
29. Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006; 174:1034-1041.
30. Giaid A, Yanagisawa M, Langleben D, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993; 328:1732-1739. (Pubitemid 23170122)
31. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995; 333:214-221.
32. Tuder RM, Cool CD, Geraci MW, et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999; 159:1925-1932.
33. Adnot S. Lessons learned from cancer may help in the treatment of pulmonary hypertension. J Clin Invest 2005; 115:1461-1463.
34. Sitbon O, Humbert M, Jais X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111:3105-3111.
35. Pietra GG, Edwards WD, Kay JM, et al. Histopathology of primary pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation 1989; 80:1198-1206.
36. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J 2006; 28:999-1004.
37. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 334:296-302.
38. 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; 112:485-491.
39. Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epo-prostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized, controlled trial. Ann Intern Med 2000; 132:425-434.
40. 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; 165:800-804. (Pubitemid 34229650)
41. Oudiz RJ, Schilz RJ, Barst RJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004; 126:420-427.
42. Tapson VF, Gomberg-Maitland M, McLaughlin VV, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006; 129:683-688.
43. Sitbon O, Manes A, Jais X, et al. Rapid switch from intravenous epoprostenol to intravenous treprostinil in patients with pulmonary arterial hypertension. J Cardiovasc Pharmacol 2007; 49:1-5.
44. Oudiz RJ, Farber HW. Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am Heart J 2009; 157:625-635.
45. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346:896-903.
46. Denton CP, Pope JE, Peter HH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related to connective tissue diseases. Ann Rheum Dis 2008; 67:1222-1228.
47. Girgis RE, Mathai SC, Krishnan JA, et al. Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 2005; 24:1626-1631.
48. Kowal-Bielecka O, Landewe R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis 2009; 68:620-628.
49. Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004; 169:441-447.
50. Girgis RE, Frost AE, Hill NS, et al. Selective endothelin A receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann Rheum Dis 2007; 66:1467-1472.
51. Benza RL, Barst RJ, Galie N, et al. Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival. Chest 2008; 134:775-782.
52. Galie N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multi-center, efficacy (ARIES) study 1 and 2. Circulation 2008; 117:3010-3019.
53. Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119:2894-2903.
54. Avouac J, Wipff J, Kahan A, Allanore Y. Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: a meta analysis of randomised controlled trials. Ann Rheum Dis 2008; 67:808-814.
55. Simonneau G, Rubin LJ, Galie N, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149:521-530.
56. Mathai SC, Girgis RE, Fisher MR, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 2007; 29:469-475.
57. Paul GA, Gibbs JS, Boobis AR, et al. Bosentan decreases the plasma concentration of sildenafil when coprescribed in pulmonary hypertension. Br J Clin Pharmacol 2005; 60:107-112.
58. Budhiraja R, Tuder RM, Hassoun PM. Endothelial dysfunction in pulmonary hypertension. Circulation 2004; 109:159-165.
59. Ghofrani HA, Seeger W, Grimminger F. Imatinib for the treatment of pulmonary arterial hypertension. N Engl J Med 2005; 353:1412-1413. (Pubitemid 41362714)
60. Patterson KC, Weissmann A, Ahmadi T, Farber HW. Imatinib mesylate in the treatment of refractory idiopathic pulmonary arterial hypertension. Ann Intern Med 2006; 145:152-153.
61. Souza R, Sitbon O, Parent F, et al. Long term imatinib treatment in pulmonary arterial hypertension. Thorax 2006; 61:736.
62. Overbeek MJ, van Nieuw Amerongen GP, Boonstra A, et al. Possible role of imatinib in clinical pulmonary veno-occlusive disease. Eur Respir J 2008; 32:232-235.
63. Ten Freyhaus H, Dumitrescu D, Bovenschulte H, et al. Significant improvement of right ventricular function by imatinib mesylate in scleroderma-associated pulmonary arterial hypertension. Clin Res Cardiol 2009; 98:265-267.
64. Dorfmuller P, Humbert M, Perros F, et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol 2007; 38:893-902.
65. Grigoryev DN, Mathai SC, Fisher MR, et al. Identification of candidate genes in scleroderma-related pulmonary arterial hypertension. Transl Res 2008; 151:197-207.
66. Schachna L, Medsger TA Jr, Dauber JH, et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006; 54:3954-3961.