Pulmonary arterial hypertension in systemic sclerosis

Autoimmunity Reviews

Volumn 9, Issue 11, 2010, Pages 761-770

  Lambova, Sevdalina ^a,b   Müller Ladner, Ulf ^b  

^a MEDICAL UNIVERSITY OF PLOVDIV   (Bulgaria)

^b JUSTUS LIEBIG UNIVERSITY   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE; AMBRISENTAN; AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; BERAPROST; BOSENTAN; BRAIN NATRIURETIC PEPTIDE; CALCITONIN GENE RELATED PEPTIDE; CD40 LIGAND; CYCLIC GMP; ILOPROST; MATRIX METALLOPROTEINASE; NITRIC OXIDE; PHOSPHATIDYLINOSITOL 3 KINASE; PLATELET DERIVED GROWTH FACTOR; PLATELET DERIVED GROWTH FACTOR A; PLATELET DERIVED GROWTH FACTOR AA; PLATELET DERIVED GROWTH FACTOR AB; PLATELET DERIVED GROWTH FACTOR B; PLATELET DERIVED GROWTH FACTOR BB; PROSTACYCLIN; PROSTAGLANDIN; SEROTONIN; SILDENAFIL; SITAXSENTAN; TADALAFIL; UNINDEXED DRUG; UNIPROST; VARDENAFIL; VASCULOTROPIN; VASOACTIVE INTESTINAL POLYPEPTIDE;

AEROBIC EXERCISE; ANEMIA; ARTHRALGIA; CHILL; CLINICAL PRACTICE; CLINICAL TRIAL; COMPUTER ASSISTED TOMOGRAPHY; CONNECTIVE TISSUE DISEASE; DIARRHEA; DISEASE ASSOCIATION; DIZZINESS; DOPPLER ECHOCARDIOGRAPHY; DRUG ANTAGONISM; DRUG DOSE INCREASE; DRUG DOSE TITRATION; DRUG EFFICACY; DRUG HALF LIFE; DRUG INHIBITION; DRUG POTENTIATION; DYSPNEA; EDEMA; ELECTROCARDIOGRAM; ERECTILE DYSFUNCTION; ERYTHEMA; FATIGUE; FEVER; FLUSHING; GENETIC PREDISPOSITION; HEADACHE; HEART ARRHYTHMIA; HEART CATHETERIZATION; HEART PALPITATION; HEART RIGHT VENTRICLE FAILURE; HUMAN; HYPOTENSION; ISOMETRIC EXERCISE; LEG EDEMA; LOADING DRUG DOSE; LUNG CIRCULATION; LUNG FIBROSIS; LUNG FUNCTION TEST; LUNG LESION; LUNG VASCULAR RESISTANCE; MOUTH PAIN; MUSCULOSKELETAL PAIN; MYALGIA; NAUSEA; NUCLEAR MAGNETIC RESONANCE; OXYGEN THERAPY; PAIN; PATHOGENESIS; PREVALENCE; PULMONARY HYPERTENSION; RASH; REVIEW; RHEUMATOID ARTHRITIS; SIDE EFFECT; SODIUM RESTRICTION; SYNCOPE; SYSTEMIC SCLEROSIS; THORAX PAIN; THORAX RADIOGRAPHY; TREATMENT RESPONSE; VOMITING;

AUTOIMMUNE DISEASES; BLOOD FLOW VELOCITY; HUMANS; HYPERTENSION; HYPERTENSION, PULMONARY; PULMONARY CIRCULATION; PULMONARY FIBROSIS; SCLERODERMA, SYSTEMIC; VASCULAR RESISTANCE;

EID: 77956187179     PISSN: 15689972     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.autrev.2010.06.006     Document Type: Review

References (95)

1. McLaughlin V.V., Archer S.L., Badesch D.B., Barst R.J., Farber H.W., Lindner J.R., 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. J AmColl Cardiol 2009, 53:1573-1619.
2. Carreira P.E. Pulmonary hypertension in autoimmune rheumatic diseases. Autoimmun Rev 2004, 3:313-320.
3. Walker U.A., Tyndall A., Czirják L., Denton C., Farge-Bancel D., Kowal-Bielecka O., et al. Clinical risk assessment of organ manifestationsin systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database. Ann Rheum Dis 2007, 66:754-763.
4. Condiffe R., Kiely D.G., Peacock A.J., Corris P.A., Gibbs J.S., Vrapi F., et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009, 179:151-157.
5. Proudman S.M., Stevens W.M., Sahhar J., Celermajer D. Pulmonary arterial hypertension in systemic sclerosis: the need for early detection and treatment. Int Med J 2007, 37:485-494.
6. Hachulla E., Gressin V., Guillevin L., Carpentier P., Diot E., Sibilia J., et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005, 52:3792-3800.
7. Frankel S.K., Brown K.K. Collagen vascular diseases of the lung. Clin Pulm Med 2006, 13:25-36.
8. Asherson R.A., Higgenbottam T.W., Dinh Xuan A.T., Khamashta M.A., Hughes GR. Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. J Rheumatol 1990, 17:1292-1298.
9. Dawson J.K., Goodson N.G., Graham D.R., Lynch M.P. Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatol 2000, 39:1320-1325.
10. Magliano M., Isenberg D.A., Hillson J. Pulmonary hypertension in autoimmune rheumatic diseases. Where are we now?. Arthritis Rheum 2002, 46:1997-2009.
11. Hesselstrand R., Scheja A., Åkesson A. Mortality and causes of death in a Swedish series of systemic sclerosis patients. Ann Rheum Dis 1998, 57:682-686.
12. Silver R.M., Medsger T.A., Bolster M.B. Systemic sclerosis and scleroderma variants: clinical aspects. Arthritis and allied conditions 2005, 1633-1680. Lippincot Williams & Wilkins. 15th ed. W.J. Koopman (Ed.).
13. Jeffery T.K., Morrell N.W. Molecular and cellular basis of pulmonary vascular remodeling in pulmonary hypertension. Prog Cardiovasc Dis 2002, 45:173-202.
14. Humbert M., Morrell N.W., Archer A.L., Stenmark K.R., MacLean M.R., Lang I.M., et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J AmColl Cardiol 2004, 43:13S-24S.
15. Grigoryev D.N., Mathai S.C., Fisher M.R., Girgis R.E., Zaiman A.L., Housten-Harris T., et al. Identification of candidate genes in scleroderma-related pulmonary arterial hypertension. Transl Res 2008, 151:197-207.
16. Tuder R.M., Cool C.D., Geraci M.W., Wang J., Abman S.H., Wright L., 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.
17. 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.
18. Morelli S., Ferri C., Di Francesco L., Baldoncini R., Carlesimo M., Bottoni U., et al. Plasma endothelin-1 levels in patients with systemic sclerosis: influence of pulmonary or systemic arterial hypertension. Ann Rheum Dis 1995, 54:730-734.
19. Montani D., Souza R., Binkert Ch., Fschli W., Simonneau G., Clozel M., et al. Endothelin-1/endothelin-3 ratio - a potential prognostic factor of pulmonary arterial hypertension. Chest 2007, 131:101-108.
20. Giaid A, Yanagisawa M, Langleben D., Michel R.P., Levy R., Shennib H., et al. Expression of endothelin-1 in the lungs of patiens with primary hypertension. N Engl J Med 1993, 328:1732-1739.
21. Rajagopalan S., Pfenninger D., Kehrer C., Chakrabarti A., Somers E., Pavlic R., et al. Increased asymmetric dimethylarginine and endothelin-1 levels in secondary Raynaud's phenomenon. Arthritis Rheum 2003, 48:1992-2000.
22. Schachna L., Wigley F.M. Targeting mediators of vascular injury in scleroderma. Curr Opin Rheumatol 2002, 14:686-693.
23. Barst R.J. A review of pulmonary arterial hypertension: role of ambrisentan. Vasc Health Risk Manag 2007, 3:11-22.
24. Boin F., Wigley M. Understanding, assessing and treating Raynaud's phenomenon. Curr Opin Rheumatol 2005, 17:752-760.
25. Mayes M.D. Endothelin and endothelin receptor antagonists in systemic rheumatic diseases. Arthritis Rheum 2003, 48:1190-1199.
26. Silver R.M. Endothelin and scleroderma lung disease. Rheumatology 2008, 47:v25-v26.
27. Betsholtz C. Biology of platelet-derived growth factors in development. Birth Defects Res C Embryo Today 2003, 69:272-285.
28. Trojanowska M. Role of PDGF in fibrotic disease and systemic sclerosis. Rheumatology 2008, 47:v2-v4.
29. Alvarez R.H., Kantarjian H.M., Cortes J.E. Biology of platelet-derived growth factor and its involvement in disease. Mayo Clin Proc 2006, 81:1241-1257.
30. Papaioannou A.I., Zakynthinos E., Kostikas K., Kiropoulos T., Koutsokera A., Ziogas A., et al. Serum VEGF levels are related to the presence of pulmonary arterial hypertension in systemic sclerosis. BMC Pulm Med 2009, 9:18-25.
31. Tuder R.M., Chacon M., Alger L., Wang J., Taraseviciene-Stewart L., Kasahara Y., et al. Expression of angiogenesis-related molecules in plexiform lesions in severe pulmonary hypertension: evidence for a process of disordered angiogenesis. J Pathol 2001, 195:367-374.
32. Allanore Y., Meune C. N-terminal pro brain natriuretic peptide: the new cornerstone of cardiovascular assessment in systemic sclerosis. Clin Exp Rheumatol 2009, 27(Suppl):59-63.
33. Allanore Y., Borerie D., Meune C., Cabanes L., Weber S., Ekindjian O.G., et al. N-terminal pro-brain natriuretic peptide as a diagnostic marker of early pulmonary hypertension in patients with systemic sclerosis and effects of calcium channel blockers. Arthritis Rheum 2003, 48:3503-3508.
34. Doran J.P., Veale D.J. Biomarkers in systemic sclerosis. Rheumatology 2008, 47:v36-v38.
35. Komura K., Fujimoto M., Matsushita T., et al. Increased serum soluble CD40 levels in patients with systemic sclerosis. J Rheumatol 2007, 34:353-358.
36. Allanore Y., Borderie D., Meune C., Lemaréchal H., Weber S., Ekindjian O.G., et al. Increased plasma soluble CD40 ligand concentrations in systemic sclerosis and association with pulmonary arterial hypertension and digital ulcers. Ann Rheum Dis 2005, 64:481-483.
37. Bunker C.B., Terenghi G., Springall D.R., Polak J.M., Dowd P.M. Deficiency of calcitonin gene-related peptide in Raynaud's phenomenon. Lancet 1990, 336:1530-1533.
38. Tjen-A-Looi S., Ekman R., Lippton H., Cary J., Keith I. CGRP and somatostatin modulate chronic pulmonary hypertension. Am J Physiol Heart Circ Physiol 1992, 263:H681-H690.
39. Bartosik I., Eskilsson J., Ekman R., Akesson A., Scheja A. Correlation between plasma concentrations of pressure in patients with systemic sclerosis calcitonin gene related peptide and pulmonary. Ann Rheum Dis 2002, 61(3):261-263.
40. Matucci-Cerinic M. Sensory neuropeptides and rheumatic disease. Rheum Dis Clin North Am 1993, 19:975-991.
41. MacLean M.R. Pulmonary hypertension and the serotonin hypothesis: where are we now?. Int J Clin Pract 2007, 61:27-31.
42. Morecroft I., Pang L., Baranowska M., Nilsen M., Loughlin L., Dempsie Y., et al. In vivo effects of a combined 5-HT1B receptor/SERT antagonist in experimental pulmonary hypertension. Cardiovasc Res 2008, 85:593-603.
43. Shah S.J., Gomberg-Maitland M., Thenappan T., Rich S. Selective serotonin reuptake inhibitors and the incidence and outcome of pulmonary hypertension. Chest 2009, 136:694-700.
44. Petkov V., Mosgoeller W., Ziesche R., Raderer M., Stiebellehner L., Vonbank K., et al. Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension. J Clin Invest 2003, 111(9):1339-1346.
45. Novotná J., Herget J. Possible role of matrix metalloproteinases in reconstruction of peripheral pulmonary arteries induced by hypoxia. Physiol Res 2002, 51:323-334.
46. Dorfmüller P., Perros F., Balabanian K., Humbert M. Inflammation in pulmonary hypertension. Eur Respir J 2003, 22:358-363.
47. Recommen- dations on the management of pulmonary hypertension in medical practice. Heart 2001, 86(Suppl I):I1-I13. British Cardiac Society Guidelines and Medical Practice Committee.
48. Humbert M., Sitbon O., Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004, 351:1425-1436.
49. Hassoun P.M. Therapies for scleroderma-related pulmonary arterial hypertension. Exp Rev Resp Med 2009, 3:187-196.
50. Chandra S., Shah S.J., Thenappan T., Archer S.L., Rich S., Gomberg-Maitland M. Carbon monoxide diffusing capacity and mortality in pulmonary arterial hypertension. J Heart Lung Transplant 2010, 29:181-187.
51. Steen V., Medsger T.A. Predictors of Iiolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003, 48:516-522.
52. Hsu V.M., Moreyra A.E., Wilson A.C., Shinnar M., Shindler D.M., Wilson J.E., et al. Assessment of pulmonary arterial hypertension in patients with systemic sclerosis: comparison of noninvasive tests with results of right-heart catheterization. J Rheumatol 2008, 35:458-465.
53. Goh N.S.L., Veeraraghavan S., Desai S.R., Cramer D., Hansell D.M., Denton C.P., et al. Bronchoalveolar lavage cellular profiles in patients with systemic sclerosis- associated interstitial lung disease are not predictive of disease progression. Arthritis Rheum 2007, 56:2005-2012.
54. Hofstee H.M., VonkNoordegraaf A., Voskuyl A.E., Dijkmans B.A., Postmus P.E., Smulders Y.M., et al. Nailfold capillary density is associated with the presence and severity of pulmonary arterial hypertension in systemic sclerosis. Ann Rheum Dis 2009, 68:191-195.
55. Lovy M., MacCarter D., Steigerwald J.C. Relationship between nailfold capillary abnormalities and organ involvement in systemic sclerosis. Arthritis Rheum 1985, 28:496-501.
56. Krasuski R.A., Warner J.J., Wang A., Harrison K., Bashore M. Inhaled nitric oxide selectively dilated pulmonary vasculature in adult patients with pulmonary hypertensionq irrespective of etiology. J Am Coll Cardiol 2000, 36:2204-2211.
57. Ricciardi M.J., Knight B.P., Martinez F.J., Rubenfire M. Inhaled nitric oxide in primary pulmonary hypertension: a safe and effective agent for predicting response to nifedipine. J Am Coll Cardiol 1998, 32:1068-1073.
58. Mathai S.C., Hassoun P.M. Therapy for pulmonary arterial hypertension associated with systemic sclerosis. Curr Opin Rheumatol 2009, 21:642-648.
59. Johnson S.R., Mehta S., Granton J.T. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J 2006, 28:999-1004.
60. Sanchez O., Sitbon O., Jais X., Simonneau G. Humbert M. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 2006, 130:182-189.
61. Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992, 327:76-81.
62. Badesch D.B. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum disease. Ann Intern Med 2000, 132:452-434.
63. McLaughlin V.V., Gaine S.P., Barst R.J., Oudiz R.J., Bourge R.C., Frost A., et al. Efficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertension. J Cardiovasc Pharmacol 2003, 41(2):293-299.
64. Bettoni L., Geri A., Airò P., Danieli E., Cavazzana I., Antonioli C., et al. Clinical systemic sclerosis therapy with iloprost: a prospective observational study of 30 patients treated for a median of 3 years. Clin Rheumatol 2002, 21(3):244-250.
65. Hoeper M.M., Spiekerkoetter E., Westerkamp V., Gatzke R., Fabel H. Intravenous iloprost for treatment failure of aerosolised iloprost in pulmonary arterial hypertension. Eur Respir J 2002, 20:339-3.
66. Della Bella S., Molteni M., Mocellin C., et al. Novel mode of action of iloprost: in vitro downregulation of endothelial cell adhesion molecules. Prostaglandins Other Lipid Mediat 2001, 65:73-83.
67. Mittag M.P., Beckheinrich U.F. Systemic sclerosis-related Raynaud's phenomenon: effects of iloprost infusion therapy on serum cytokine, growth factor and soluble adhesion molecule levels. Acta Derm Venereol 2001, 81:294-297.
68. Scorza R., Caronni M., Mascagni B., et al. Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud's phenomenon. A randomized controlled study. Clin Exp Rheumatol 2001, 19:503-508.
69. Olschewski H., Simonneau G., Galiè N., Higenbottam T., Naeije R., Rubin L.J., et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002, 347:322-329.
70. Ivy D.D., Doran A.K., Smith K.J., Mallory G.B., Beghetti M., Barst R.J., et al. Short- and long- term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol 2008, 51:161-169.
71. Galie N., Humbert M., Vachiery J.L., Vizza C.D., Kneussl M., Manes A., et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebocontrolled trial. J Am Coll Cardiol 2002, 39(9):1496-1502.
72. Saji T., Ozawa Y., Ishikita T., Matsuura H., Matsuo N. Short term hemodinamic effect of a new oral PgI2 analogue, Beraprost, in pulmonary and secondary pulmonary hypertension. Am J Cardiol 1996, 78(2):244-247.
73. Korn J.H., Mayes M. Matucci Cerinic M, Rainisio M, Pope J, Hachulla E et al. Digital ulcers in systemic sclerosis prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004, 50:3985-3993.
74. Rubin L.J., Badesch D.B., Barst R.J., Galie N., Black C.M., Keogh A., et al. Bosentan therapy for pulmonary hypertension. N Engl J Med 2002, 346:896-903.
75. Galie N., Rubin L.J., Koeper M.M., Jansa P., Al-Hiti H., Meyer G.M.B., et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomized controlled trial. Lancet 2008, 371:2093-2100.
76. Denton C.P., Humbert M., Rubin L., Black C.M. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 2006, 65:1336-1340.
77. Waxman A.B. A review of sitaxsentan sodium in patients with pulmonary arterial hypertension. Vasc Health Risk Manag 2007, 3:151-157.
78. Galié N., Badesch D., Oudiz R., Simonneau G., McGoon M.D., Keogh A.M., et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005, 46(3):529-535.
79. Vatter H., Seifert V. Ambrisentan, a non-peptide endothelin receptor antagonist. Cardiovasc Drug Rev 2006, 24:63-76.
80. Shenoy P., Agarwal V. Phosphodiesterase inhibitors in the management of autoimmune disease. Autoimmune Rev 2010, 9(7):511-515.
81. Galie N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005, 353:2148-2157.
82. Hoeper M.M., Welte T. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2006, 354:1091-1093.
83. Kamata Y., Kamimura T., Iwamoto M., Minota S. Comparable effects of sildenafil citrate and alprostadil on severe Raynaud's phenomenon in a patient with systemic sclerosis. Clin Exp Dermatol 2005, 30:451.
84. Kumana C.R., Cheung G.T.Y., Lau C.S. Severe digital ischaemia treated with phosphodiesterase inhibitors. Ann Rheum Dis 2004, 63:1522-1524.
85. Rosenkranz S., Diet F., Karasch T., Weihrauch J., Wassermann K., Erdmann E. Sildenafil improved pulmonary hypertension and peripheral blood flow in a patient with scleroderma-associated lung fibrosis and Raynaud's phenomenon. Ann Int Med 2003, 139:871-873.
86. Baumhaekel M., Scheffler P., Boehm M. Use of tadalafil in patient with secondary Raynaud's phenomenon not responding to sildenafil. Microvasc Res 2005, 69:178-179.
87. Ghofrani H.A., Voswinckel R., Reichenberger F., Olschewski H., Haredza P., Karada B., et al. Differences in hemodynamic and oxygenation responses to three different phoshodiesterase-5 inhibitors in patients with pulmonary arterial hypertension. J Am Coll Cardiol 2004, 44:1488-75.
88. Distler J.H.W., Distler O. Intracellular tyrosine kinase as a novel targets for anti-fibrotic therapy in systemic sclerosis. Rheumatology 2008, 47:v10-v11.
89. Schermuly R.T., Dony E., Ghofrani H.A., Pullamsetti S., Savai R., Roth M., et al. Reversal 1037of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest 2005, 115:2811-2821.
90. Beyer C, Distler JHW, Distler O. Are tyrosine kinase inhibitors promising for the treatment of systemic sclerosis and other fibrotic diseases?
91. Li M., Li Z., Sun X. Statins supress MMP2 secretion via inactivation Rho/ROCK pathway in pulmonary vascular smooth muscles cells. Eur J Pharmacol 2008, 591:219-223.
92. Wang X.X., Zhang F., Shang Y., Zhu J., Xie X., Tao Q., et al. Transplantation of autologous endothelial progenitor cells may be beneficial in patients with idiopathic pulmonary arterial hypertension. J Am Coll Cardiol 2007, 49:1566-1571.
93. Koh E.T., Lee P., Gladman D., Abu-Shakram M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996, 35:989-993.
94. Fisher M.R., Mathai S.C., Champion H.C., Girgis R.E., Housten-Harris T., Hummers L., et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006, 54:3043-3050.
95. Ranque B., Mouthon L. Geoepidemiology of systemic sclerosis. Autoimmune Rev 2010, 9:A311-A318.