Pulmonary arterial hypertension associated with systemic sclerosis

Expert Review of Respiratory Medicine

Volumn 5, Issue 2, 2011, Pages 267-279

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

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

Author keywords

diagnosis; pulmonary hypertension; systemic sclerosis; therapy

Indexed keywords

AUTOANTIBODY; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; IMATINIB; PHOSPHODIESTERASE V INHIBITOR; PROSTACYCLIN; PROSTAGLANDIN; SILDENAFIL; TADALAFIL;

ANTICOAGULATION; AUTOIMMUNITY; CELL PROLIFERATION; CLINICAL FEATURE; DISEASE ASSOCIATION; DRUG EFFICACY; GASTROINTESTINAL TRACT; HEREDITY; HUMAN; LUNG BLOOD VESSEL; LUNG TRANSPLANTATION; OUTCOME ASSESSMENT; PATHOGENESIS; PREVALENCE; PULMONARY HYPERTENSION; REVIEW; RISK ASSESSMENT; RISK FACTOR; SCLERODERMA; SCREENING TEST; SURVIVAL RATE; SYSTEMIC SCLEROSIS; TREATMENT RESPONSE;

EID: 79955383532     PISSN: 17476348     EISSN: 17476356     Source Type: Journal    
DOI: 10.1586/ers.11.18     Document Type: Review

References (138)

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. J. Am. Coll. Cardiol. 53(17), 1573-1619 (2009).
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. 62(11), 1088-1093 (2003). (Pubitemid 37323574)
3. 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. 52(12), 3792-3800 (2005). (Pubitemid 41798217)
4. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann. Rheum. Dis. 66(7), 940-944 (2007). (Pubitemid 46999798)
5. Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 123(2), 344-350 (2003). (Pubitemid 36269414)
6. Fisher MR, Mathai SC, Champion HC et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 54(9), 3043-3050 (2006). (Pubitemid 44497786)
7. McLaughlin VV, Presberg KW, Doyle RL et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126(Suppl. 1), 78S-92S (2004). (Pubitemid 38932344)
8. Jimenez SA, Derk CT. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann. Intern. Med. 140(1), 37-50 (2004). (Pubitemid 38055920)
9. Tan FK. Systemic sclerosis: the susceptible host (genetics and environment). Rheum. Dis. Clin. North Am. 29(2), 211-237 (2003). (Pubitemid 36765118)
10. Arnett FC, Cho M, Chatterjee S, Aguilar MB, Reveille JD, Mayes MD. Familial occurrence frequencies and relative risks for systemic sclerosis (scleroderma) in three United States cohorts. Arthritis Rheum. 44(6), 1359-1362 (2001). (Pubitemid 32537534)
11. Feghali-Bostwick C, Medsger TA Jr, Wright TM. Analysis of systemic sclerosis in twins reveals low concordance for disease and high concordance for the presence of antinuclear antibodies. Arthritis Rheum. 48(7), 1956-1963 (2003). (Pubitemid 36828691)
12. Nietert PJ, Silver RM. Systemic sclerosis: environmental and occupational risk factors. Curr. Opin. Rheumatol. 12(6), 520-526 (2000).
13. Mayes MD. Scleroderma epidemiology. Rheum. Dis. Clin. North Am. 29(2), 239-254 (2003). (Pubitemid 36765119)
14. Mayes MD, Lacey JV Jr, Beebe-Dimmer J et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 48(8), 2246-2255 (2003). (Pubitemid 36959206)
15. Allcock RJ, Forrest I, Corris PA, Crook PR, Griffiths ID. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology (Oxford) 43(5), 596-602 (2004). (Pubitemid 38628190)
16. Tamaki T, Mori S, Takehara K. Epidemiological study of patients with systemic sclerosis in Tokyo. Arch. Dermatol. Res. 283(6), 366-371 (1991).
17. Steen VD, Oddis CV, Conte CG, Janoski J, Casterline GZ, Medsger TA Jr. Incidence of systemic sclerosis in Allegheny County, Pennsylvania. A twenty-year study of hospital-diagnosed cases, 1963-1982. Arthritis Rheum. 40(3), 441-445 (1997).
18. Le Guern V, Mahr A, Mouthon L, Jeanneret D, Carzon M, Guillevin L. Prevalence of systemic sclerosis in a French multi-ethnic county. Rheumatology (Oxford) 43(9), 1129-1137 (2004). (Pubitemid 39232818)
19. Chifflot H, Fautrel B, Sordet C, Chatelus E, Sibilia J. Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin. Arthritis Rheum. 37(4), 223-235 (2008).
20. Laing TJ, Gillespie BW, Toth MB et al. Racial differences in scleroderma among women in Michigan. Arthritis Rheum. 40(4), 734-742 (1997). (Pubitemid 27157581)
21. LeRoy EC, Black C, Fleischmajer R et al. Scleroderma (systemic sclerosis), classification, subsets and pathogenesis. J. Rheumatol. 15(2), 202-205 (1988). (Pubitemid 18073738)
22. LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J. Rheumatol. 28(7), 1573-1576 (2001).
23. Johnson DA, Drane WE, Curran J et al. Pulmonary disease in progressive systemic sclerosis. A complication of gastroesophageal reflux and occult aspiration? Arch. Intern. Med. 149(3), 589-593 (1989). (Pubitemid 19092856)
24. Savarino E, Bazzica M, Zentilin P et al. Gastroesophageal reflux and pulmonary fibrosis in scleroderma: a study using pH-impedance monitoring. Am. J. Respir. Crit. Care Med. 179(5), 408-413 (2009).
25. Roumm AD, Medsger TA Jr. Cancer and systemic sclerosis. An epidemiologic study. Arthritis Rheum. 28(12), 1336-1340 (1985).
26. Chatterjee S, Dombi GW, Severson RK, Mayes MD. Risk of malignancy in scleroderma: a population-based cohort study. Arthritis Rheum. 52(8), 2415-2424 (2005). (Pubitemid 41117427)
27. Olsen NJ, King LE Jr, Park JH. Muscle abnormalities in scleroderma. Rheum. Dis. Clin. North Am. 22(4), 783-796 (1996). (Pubitemid 26396051)
28. Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br. J. Rheumatol. 37(12), 1320-1323 (1998).
29. Vitali C, Viegi G, Tassoni S et al. Lung function abnormalities in different connective tissue diseases. Clin. Rheumatol. 5(2), 181-188 (1986).
30. Lang B, Ortlieb H, Meske S, Hauke G, Peter HH. Progressive systemic sclerosis presenting with spontaneous pneumothorax. J. Rheumatol. 16(2), 254-256 (1989).
31. Varga J. Systemic sclerosis: an update. Bull. NYU Hosp. Jt Dis. 66(3), 198-202 (2008).
32. Morelli S, Barbieri C, Sgreccia A et al. Relationship between cutaneous and pulmonary involvement in systemic sclerosis. J. Rheumatol. 24(1), 81-85 (1997). (Pubitemid 27059862)
33. Kane GC, Varga J, Conant EF, Spirn PW, Jimenez S, Fish JE. Lung involvement in systemic sclerosis (scleroderma), relation to classification based on extent of skin involvement or autoantibody status. Respir. Med. 90(4), 223-230 (1996). (Pubitemid 26130211)
34. McNearney TA, Reveille JD, Fischbach M et al. Pulmonary involvement in systemic sclerosis: associations with genetic, serologic, sociodemographic, and behavioral factors. Arthritis Rheum. 57(2), 318-326 (2007). (Pubitemid 47164096)
35. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 43(11), 2437-2444 (2000).
36. Battle RW, Davitt MA, Cooper SM et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest 110(6), 1515-1519 (1996). (Pubitemid 26425153)
37. Stupi AM, Steen VD, Owens GR, Barnes EL, Rodnan GP, Medsger TA Jr. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum. 29(4), 515-524 (1986). (Pubitemid 16118701)
38. Humbert M, Sitbon O, Chaouat A et al. Pulmonary arterial hypertension in France: results from a national registry. Am. J. Respir. Crit. Care Med. 173(9), 1023-1030 (2006).
39. Avouac J, Airo P, Meune C et al. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J. Rheumatol. 37(11), 2290-2298 (2010).
40. Badesch DB, Raskob GE, Elliott CG et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 137(2), 376-387 (2010).
41. Overbeek MJ, Vonk MC, Boonstra A et al. Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. Eur. Respir. J. 34(2), 371-379 (2009).
42. 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. 38(6), 893-902 (2007). (Pubitemid 46735956)
43. LeRoy EC. Systemic sclerosis. A vascular perspective. Rheum. Dis. Clin. North Am. 22(4), 675-694 (1996).
44. Muller-Ladner U, Distler O, Ibba-Manneschi L, Neumann E, Gay S. Mechanisms of vascular damage in systemic sclerosis. Autoimmunity 42(7), 587-595 (2009).
45. Sgonc R, Gruschwitz MS, Boeck G, Sepp N, Gruber J, Wick G. Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95. Arthritis Rheum. 43(11), 2550-2562 (2000).
46. Cerinic MM, Valentini G, Sorano GG et al. Blood coagulation, fibrinolysis, and markers of endothelial dysfunction in systemic sclerosis. Semin. Arthritis Rheum. 32(5), 285-295 (2003). (Pubitemid 36459278)
47. Denton CP, Bickerstaff MC, Shiwen X et al. Serial circulating adhesion molecule levels reflect disease severity in systemic sclerosis. Br. J. Rheumatol. 34(11), 1048-1054 (1995). (Pubitemid 26013507)
48. Distler O, Del Rosso A, Giacomelli R et al. Angiogenic and angiostatic factors in systemic sclerosis: increased levels of vascular endothelial growth factor are a feature of the earliest disease stages and are associated with the absence of fingertip ulcers. Arthritis Res. 4(6), R11 (2002).
49. Choi JJ, Min DJ, Cho ML et al. Elevated vascular endothelial growth factor in systemic sclerosis. J. Rheumatol. 30(7), 1529-1533 (2003).
50. Hebbar M, Peyrat JP, Hornez L, Hatron PY, Hachulla E, Devulder B. Increased concentrations of the circulating angiogenesis inhibitor endostatin in patients with systemic sclerosis. Arthritis Rheum. 43(4), 889-893 (2000).
51. Hummers LK, Hall A, Wigley FM, Simons M. Abnormalities in the regulators of angiogenesis in patients with scleroderma. J. Rheumatol. 36(3), 576-582 (2009).
52. Grigoryev DN, Mathai SC, Fisher MR et al. Identification of candidate genes in scleroderma-related pulmonary arterial hypertension. Transl. Res. 151(4), 197-207 (2008).
53. Okano Y, Steen VD, Medsger TA Jr. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum. 35(1), 95-100 (1992).
54. Rosen A, Casciola-Rosen L. Autoantigens in systemic autoimmunity: critical partner in pathogenesis. J. Intern. Med. 265(6), 625-631 (2009).
55. Tamby MC, Chanseaud Y, Humbert M et al. Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. Thorax 60(9), 765-772 (2005). (Pubitemid 41297897)
56. Grigolo B, Mazzetti I, Meliconi R et al. Anti-topoisomerase II a autoantibodies in systemic sclerosis-association with pulmonary hypertension and HLA-B35. Clin. Exp. Immunol. 121(3), 539-543 (2000).
57. Nicolls MR, Taraseviciene-Stewart L, Rai PR, Badesch DB, Voelkel NF. Autoimmunity and pulmonary hypertension: a perspective. Eur. Respir. J. 26(6), 1110-1118 (2005). (Pubitemid 41829945)
58. Mihai C, Tervaert JW. Anti-endothelial cell antibodies in systemic sclerosis. Ann. Rheum. Dis. 69(2), 319-324 (2010).
59. Tamby MC, Humbert M, Guilpain P et al. Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Eur. Respir. J. 28(4), 799-807 (2006). (Pubitemid 46332002)
60. Tamby MC, Servettaz A, Tamas N et al. IgG from patients with systemic sclerosis bind to DNA antitopoisomerase 1 in normal human fibroblasts extracts. Biologics 2(3), 583-591 (2008).
61. Terrier B, Tamby MC, Camoin L et al. Identification of target antigens of antifibroblast antibodies in pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 177(10), 1128-1134 (2008). (Pubitemid 351717572)
62. Riemekasten G, Philippe A, Nather M et al. Involvement of functional autoantibodies against vascular receptors in systemic sclerosis. Ann. Rheum. Dis. 70(3), 530-536 (2010).
63. Sanchez O, Sitbon O, Jais X, Simonneau G, Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130(1), 182-189 (2006). (Pubitemid 44116858)
64. Jais X, Launay D, Yaici A et al. Immunosuppressive therapy in lupus-and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum. 58(2), 521-531 (2008). (Pubitemid 351294886)
65. Austin ED, Loyd JE. Genetics and mediators in pulmonary arterial hypertension. Clin. Chest Med. 28(1), 43-57, VII-VIII (2007). (Pubitemid 46343757)
66. Trembath RC, Thomson JR, Machado RD et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N. Engl. J. Med. 345(5), 325-334 (2001). (Pubitemid 32703235)
67. Girerd B, Montani D, Coulet F et al. Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation. Am. J. Respir. Crit. Care Med. 181(8), 851-861 (2010).
68. Tew MB, Arnett FC, Reveille JD, Tan FK. Mutations of bone morphogenetic protein receptor type II are not found in patients with pulmonary hypertension and underlying connective tissue diseases. Arthritis Rheum. 46(10), 2829-2830 (2002). (Pubitemid 36118956)
69. Morse J, Barst R, Horn E, Cuervo N, Deng Z, Knowles J. Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations. J. Rheumatol. 29(11), 2379-2381 (2002). (Pubitemid 35266029)
70. Wipff J, Kahan A, Hachulla E et al. Association between an endoglin gene polymorphism and systemic sclerosis-related pulmonary arterial hypertension. Rheumatology (Oxford) 46(4), 622-625 (2007). (Pubitemid 46523307)
71. Mattuzzi S, Barbi S, Carletto A et al. Association of polymorphisms in the IL1B and IL2 genes with susceptibility and severity of systemic sclerosis. J. Rheumatol. 34(5), 997-1004 (2007). (Pubitemid 46774845)
72. Dieude P, Guedj M, Wipff J et al. Association of the TNFAIP3 rs5029939 variant with systemic sclerosis in the European Caucasian population. Ann. Rheum. Dis. 69(11), 1958-1964 (2010).
73. 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. 165(6), 800-804 (2002). (Pubitemid 34229650)
74. 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. 66(11), 1467-1472 (2007). (Pubitemid 350032654)
75. MacDonald SL, Rubens MB, Hansell DM et al. Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT. Radiology 221(3), 600-605 (2001). (Pubitemid 33101748)
76. Mathai SC, Hummers LK, Champion HC et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum. 60(2), 569-577 (2009).
77. 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. 179(2), 151-157 (2009).
78. Campo A, Mathai SC, Le Pavec J et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 182(2), 252-260 (2010).
79. Steen V, Medsger TA Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum. 48(2), 516-522 (2003). (Pubitemid 36278021)
80. Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC. Age and risk of pulmonary arterial hypertension in scleroderma. Chest 124(6), 2098-2104 (2003). (Pubitemid 38010575)
81. Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J. Rheumatol. 33(2), 269-274 (2006). (Pubitemid 43209302)
82. 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. 58(1), 284-291 (2008).
83. Steen VD. The many faces of scleroderma. Rheum. Dis. Clin. North Am. 34(1), 1-15; V (2008). (Pubitemid 351329460)
84. Campo A, Mathai SC, Le Pavec J et al. Outcomes of hospitalization for right heart failure in pulmonary arterial hypertension. Eur. Respir. J. DOI: 10.1183/09031936.00148310 (2011) (Epub ahead of print).
85. Boueiz A, Mathai SC, Hummers LK, Hassoun PM. Cardiac complications of systemic sclerosis: recent progress in diagnosis. Curr. Opin. Rheumatol. 22(6), 696-703 (2010).
86. Mathai SC, Bueso M, Hummers LK et al. Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. Eur. Respir. J. 35(1), 95-104 (2010).
87. 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. 177(12), 1364-1369 (2008). (Pubitemid 351860914)
88. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron. Artery Dis. 16(1), 13-18 (2005). (Pubitemid 40279592)
89. Overbeek MJ, Lankhaar JW, Westerhof N et al. Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. Eur. Respir. J. 31(6), 1160-1166 (2008).
90. Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. 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. 52(7), 2125-2132 (2005). (Pubitemid 40994336)
91. Mangat P, Conron M, Gabbay E, Proudman SM. Scleroderma lung disease, variation in screening, diagnosis and treatment practices between rheumatologists and respiratory physicians. Intern. Med. J. 40(7), 494-502 (2010).
92. Huscher D, Pittrow D, Distler O et al. Interactions between rheumatologists and cardio-/pulmonologists in the assessment and use of outcome measures in pulmonary arterial hypertension related to systemic sclerosis. Clin. Exp. Rheumatol. 28(2 Suppl. 58), S47-S52 (2010).
93. 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. 161(2 Pt 1), 487-492 (2000). (Pubitemid 30129457)
94. Avouac J, Kowal-Bielecka O, Pittrow D et al. Validation of the 6 min walk test according to the OMERACT filter: a systematic literature review by the EPOSS-OMERACT group. Ann. Rheum. Dis. 69(7), 1360-1363 (2010).
95. Gilbert C, Brown MC, Cappelleri JC, Carlsson M, McKenna SP. Estimating a minimally important difference in pulmonary arterial hypertension following treatment with sildenafil. Chest 135(1), 137-142 (2009).
96. Galie N, Ghofrani HA, Torbicki A et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N. Engl. J. Med. 353(20), 2148-2157 (2005). (Pubitemid 41653106)
97. Garin MC, Highland KB, Silver RM, Strange C. Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J. Rheumatol. 36(2), 330-336 (2009).
98. 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. 40(4), 780-788 (2002). (Pubitemid 34925627)
99. Provencher S, Sitbon O, Humbert M, Cabrol S, Jais X, Simonneau G. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur. Heart J. 27(5), 589-595 (2006).
100. Distler O, Behrens F, Pittrow D et al. Defining appropriate outcome measures in pulmonary arterial hypertension related to systemic sclerosis: a Delphi consensus study with cluster analysis. Arthritis Rheum. 59(6), 867-875 (2008). (Pubitemid 351847571)
101. 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. 160(2), 487-492 (1999). (Pubitemid 29380662)
102. Fijalkowska A, Kurzyna M, Torbicki A et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 129(5), 1313-1321 (2006).
103. 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. 27(12), 1485-1494 (2006). (Pubitemid 43998084)
104. Forfia PR, Fisher MR, Mathai SC et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 174(9), 1034-1041 (2006). (Pubitemid 44684453)
105. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N. Engl. J. Med. 333(4), 214-221 (1995).
106. Giaid A, Yanagisawa M, Langleben D et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N. Engl. J. Med. 328(24), 1732-1739 (1993). (Pubitemid 23170122)
107. 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. 159(6), 1925-1932 (1999). (Pubitemid 29275065)
108. Adnot S. Lessons learned from cancer may help in the treatment of pulmonary hypertension. J. Clin. Invest. 115(6), 1461-1463 (2005). (Pubitemid 40814651)
109. Sitbon O, Humbert M, Jais X et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 111(23), 3105-3111 (2005). (Pubitemid 40847788)
110. 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 80(5), 1198-1206 (1989). (Pubitemid 19282508)
111. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur. Respir. J. 28(5), 999-1004 (2006). (Pubitemid 46331971)
112. 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. 334(5), 296-302 (1996).
113. 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. 112(7), 485-491 (1990). (Pubitemid 20100285)
114. Badesch DB, Tapson VF, McGoon MD et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann. Intern. Med. 132(6), 425-434 (2000). (Pubitemid 30149039)
115. Oudiz RJ, Schilz RJ, Barst RJ et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 126(2), 420-427 (2004). (Pubitemid 39093426)
116. Oudiz RJ, Farber HW. Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am. Heart J. 157(4), 625-635 (2009).
117. 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. 55(18), 1915-1922 (2010).
118. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N. Engl. J. Med. 346(12), 896-903 (2002). (Pubitemid 34984638)
119. 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. 67(9), 1222-1228 (2008).
120. Girgis RE, Mathai SC, Krishnan JA, Wigley FM, Hassoun PM. 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. 24(10), 1626-1631 (2005). (Pubitemid 41396463)
121. Barst RJ, Langleben D, Frost A et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 169(4), 441-447 (2004). (Pubitemid 38187114)
122. Galie N, Badesch D, Oudiz R et al. Ambrisentan therapy for pulmonary arterial hypertension. J. Am. Coll. Cardiol. 46(3), 529-535 (2005). (Pubitemid 41040298)
123. Galie N, Brundage BH, Ghofrani HA et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 119(22), 2894-2903 (2009).
124. 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. 67(6), 808-814 (2008). (Pubitemid 351829381)
125. 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. 149(8), 521-530 (2008).
126. Mathai SC, Girgis RE, Fisher MR et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur. Respir. J. 29(3), 469-475 (2007). (Pubitemid 46372092)
127. Paul GA, Gibbs JS, Boobis AR, Abbas A, Wilkins MR. Bosentan decreases the plasma concentration of sildenafil when coprescribed in pulmonary hypertension. Br. J. Clin. Pharmacol. 60(1), 107-112 (2005). (Pubitemid 40942789)
128. Ghofrani HA, Seeger W, Grimminger F. Imatinib for the treatment of pulmonary arterial hypertension. N. Engl. J. Med. 353(13), 1412-1413 (2005). (Pubitemid 41362714)
129. Patterson KC, Weissmann A, Ahmadi T, Farber HW. Imatinib mesylate in the treatment of refractory idiopathic pulmonary arterial hypertension. Ann. Intern. Med. 145(2), 152-153 (2006). (Pubitemid 46780594)
130. Souza R, Sitbon O, Parent F, Simonneau G, Humbert M. Long term imatinib treatment in pulmonary arterial hypertension. Thorax 61(8), 736 (2006). (Pubitemid 44211786)
131. Overbeek MJ, van Nieuw Amerongen GP, Boonstra A, Smit EF, Vonk-Noordegraaf A. Possible role of imatinib in clinical pulmonary veno-occlusive disease. Eur. Respir. J. 32(1), 232-235 (2008).
132. ten Freyhaus H, Dumitrescu D, Bovenschulte H, Erdmann E, Rosenkranz S. Significant improvement of right ventricular function by imatinib mesylate in scleroderma-associated pulmonary arterial hypertension. Clin. Res. Cardiol. 98(4), 265-267 (2009).
133. Ghofrani HA, Morrell NW, Hoeper MM et al. Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy. Am. J. Respir. Crit. Care Med. 182(9), 1171-1177 (2010).
134. Reich N, Maurer B, Akhmetshina A et al. The transcription factor Fra-2 regulates the production of extracellular matrix in systemic sclerosis. Arthritis Rheum. 62(1), 280-290 (2010).
135. Maurer B, Busch N, Jungel A et al. Transcription factor fos-related antigen-2 induces progressive peripheral vasculopathy in mice closely resembling human systemic sclerosis. Circulation 120(23), 2367-2376 (2009).
136. 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. 54(12), 3954-3961 (2006). (Pubitemid 44955899)
137. Hassoun PM. Therapies for scleroderma-related pulmonary arterial hypertension. Expert Rev. Respir. Med. 3(2), 187-196 (2009).
138. Mathai SC, Hassoun PM. Therapy for pulmonary arterial hypertension associated with systemic sclerosis. Curr. Opin. Rheumatol. 21, 642-648 (2009).