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DOI: 10.1055/s-2003-41089
Pulmonary Hypertension in the Collagen Vascular Diseases
Publication History
Publication Date:
01 August 2003 (online)
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ABSTRACT
Pulmonary hypertension is increasingly recognized as a complication of the collagen vascular diseases. Both isolated and secondary forms of the disease may be appreciated. Pulmonary hypertension is observed with high prevalence in scleroderma and mixed connective tissue disease. It is less commonly seen in systemic lupus erythematosus, and is a rare clinical finding in rheumatoid arthritis. The diagnosis and treatment of pulmonary hypertension associated with the collagen vascular diseases mirror diagnosis and treatment for pulmonary hypertension of any etiology. Vasodilator therapy may play a particularly important role in patients with the isolated form of disease, which is most frequently associated with scleroderma.
KEYWORDS
Pulmonary hypertension - collagen vascular disease - scleroderma - mixed connective tissue disease - systemic lupus erythematosus
REFERENCES
- 1 Roncoroni A J, Alvarez C, Molinas F. Plexogenic arteriopathy associated with pulmonary vasculitis in systemic lupus erythematosus. Respiration . 1992; 59 52-56
- 2 Murin S, Wiedemann H P, Matthay R A. Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med . 1998; 19 641-665
- 3 Kishida Y, Kanai Y, Kiramochi S. Pulmonary venoocclusive disease in a patient with systemic lupus erythematosus. J Rheumatol . 1993; 20 2161-2162
- 4 Colby T V. Pulmonary pathology in patients with systemic autoimmune diseases. Clin Chest Med . 1998; 19 587-612
- 5 Palevsky H I, Gurughagavatula I. Pulmonary hypertension in collagen vascular disease. Comp Ther . 1999; 25 133-143
- 6 LeRoy E C. Pulmonary hypertension: the bete noire of the diffuse connective tissue diseases. Am J Med . 1991; 90 539-540
- 7 LeRoy E C, Black C, Fleischmajer R. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol . 1988; 15 202-205
- 8 Nishimaki T, Aotsuka S, Kondo H. et al . Immunological analysis of pulmonary hypertension in connective tissue diseases. J Rheumatol . 1999; 26 2357-2362
- 9 Alarcon-Segovia D, Eeleze M, Oria C V. Antiphospholipid antibodies and the antiphospholipid syndrome in systemic lupus erythematosus: a prospective analysis of 500 consecutive patients. Medicine . 1989; 68 353-365
- 10 Asherson R A, Hackett D, Gharavi A E, Harris E N, Kennedy H G, Hughes G R. Pulmonary hypertensiion in systemic lupus erythematosus: a report of three cases. J Rheumatol . 1986; 13 416-420
- 11 Asherson R A, Cervera R. Review: antiphospholipid antibodies and the lung. J Rheumatol . 1995; 22 62-66
- 12 Asherson R A, Higenbottam T W, Dinh Xuan T A. Pulmonary hypertension in a lupus clinic: Experience with twenty-four patients. J Rheumatol . 1990; 17 1292
- 13 Gussin H AE, Ignat G P, Varga J, Teodorescu M. Anti-topoisomerase I (Anti-Scl-70) antibodies in patients with systemic lupus erythematosus. Arthr Rheum . 2001; 44 376-383
- 14 Yoshio T, Masuyama J, Sumiya M. Antiendothelial cell antibodies and their relation to pulmonary hypertension insystemic lupus erythematosus. J Rheumatol . 1994; 21 2058-2063
- 15 Quismorio F P, Sharma O, Koss M. Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus. Semin Arthritis Rheum . 1984; 13 349-359
- 16 Yeo P PB, Sinniah R. Lupus cor pulmonale with electron microscope and immunofluorescent antibody studies. Ann Rheum Dis . 1975; 34 457-458
- 17 Sanchez O, Humberg M, Sitbon O, Simonneau G. Treatment of pulmonary hypertension secondary to connective tissue diseases. Thorax . 1999; 54 273-277
- 18 Sanchez O, Humbert M, Sitbon O, Nunes H, Garcia G, Simonneau G. Pulmonary hypertension associated with connective tissue diseases. Revue de Medecine Interne . 2002; 23 41-54
- 19 Goupille P, Fauchier L, Babuty D. Precapillary pulmonary hypertension dramatically improved with high doses of corticosteroids during systemic lupus erythematosus. J Rheumatol . 1994; 21 1976-1977
- 20 Groen H, Bootsma H, Postma D S. Primary pulmonary hypertension in a patient with systemic lupus erythematosus: partial improvement with cyclophosphamide. J Rheumatol . 1993; 20 1055-1057
- 21 Mahowald M L, Weir E K, Ridley D J. Pulmonary hypertension in systemic lupus ereythematosus: effect of vasodilators on pulmonary hemodynamics. J Rheumatol . 1985; 12 773-777
- 22 Minai O A, Dweik R A, Arroliga A C. Manifestations of scleroderma pulmonary disease. Clin Chest Med . 1998; 713-731
- 23 Battle R W, Davitt M A, Cooper S M. et al . Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest . 1996; 110 1515-1519
- 24 Bolster M B, Silver R M. Lung disease in systemic sclerosis (scleroderma). Bailliere's Clin Rheum . 1993; 7 79-97
- 25 Coghlan J G, Mukerjee D. The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology. Curr Opinion Rheumatol . 2001; 13 495-499
- 26 Stupi A, Steen V, Owens G. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum . 1986; 29 515-524
- 27 Ungerer R G, Tashkin D P, Furst D. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis. Am J Med . 1983; 75 65-74
- 28 D'Angelo W A, Fries J F, Masi A T. Pathologic observations in systemic sclerosis (scleroderma). Am J Med . 1969; 46 428-440
- 29 Owens G R, Follansbee W P. Cardiopulmonary manifestations of systemic sclerosis. Chest . 1987; 91 118-127
- 30 Yousem S A. The pulmonary pathologic manifestations of the CREST syndrome. Hum Pathol . 1990; 21 467-474
- 31 Kerin K, Yost J H. Advances in the diagnosis and management of scleroderma-related vascular complications. Comp Ther . 1998; 24 574-581
- 32 Steen V D, Powell D L, Medsger Jr A T. Clinical correlations and prognosis based on serum autoantibodies in patients with systemic sclerosis. Arthritis Rheum . 1988; 31 196-203
- 33 Denton C P, Cailes J B, Phillips G D, Wells A U, Black C M, Du Bois M R. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis. British J Rheumatol . 1997; 36 239-243
- 34 Murata I, Takenaka K, Yoshinoya S. et al . Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders. Chest . 1997; 111 36-43
- 35 Wallman L L, Penny R, Williamson D J. Pulmonary vascular aspects of systemic sclerosis. Aust NZ J Med . 1996; 26 150-153
- 36 Christman B W, McPherson C D, Newman J H. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med . 1992; 327 70-75
- 37 Vancheeswaran R, Magoulas I, Efrat G. Circulating endothelin-1 in systemic sclerosis subsets: a marker of fibrosis or vascular dysfunction?. J Rheumatol . 1994; 21 1838-1844
- 38 Stewart D J, Levy R D, Cernacek P, Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease?. Ann Intern Med . 1991; 114 464-469
- 39 Giaid A, Yanagisawa M, Langleben D. Expression of endothelin-1 in the lungs of patients with pulmonary hypertensiion. N Engl J Med . 1993; 328 1732-1739
- 40 Duchman S M, Thohan V, Kaira D. Endothelin-1: a new target of therapeutic intervention for the treatment of heart failure. Curr Opin Cardiol . 2002; 15 136-140
- 41 Harvey A O, Shulman L E, Tulmulty P A, Conley C L, Schoenrich E H. Systemic lupus erythematosus: review of the literature and clinical anaysis of 138 cases. Medicine . 1954; 33 291-437
- 42 Cervera R, Khamashta M A, Font J. et al . Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. Medicine . 1972; 72 113-124
- 43 Winslow T M, Ossipov M A, Fazio G P, Simonson J S, Redberg R F, Schiller N B. Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus. Am Heart J . 1995; 129 510-515
- 44 Simonson J S, Schiller N B, Petri M, Hellmann D B. Pulmonary hypertension in systemic lupus erythematosus. J Rheumatol . 1989; 16 918-925
- 45 Grigor R, Edmonds J, Lewkonia R. Systemic lupus erythematosus: a prospective analysis. Ann Rheum Dis . 1978; 37 121-128
- 46 Gross M, Esterly J R, Earle R H. Pulmonary alterations in systemic lupus erythematosus. Am Rev Respir Dis . 1972; 105 572-577
- 47 Miyata M, Suzuki K, Sakuma F. Anticardiolipin antibodies are associated wirth pulmonary hypertension in patients with mixed connective tissue disease or systemic lupus erythematosus. Int Arch Allergy Immunol . 1993; 100 351-354
- 48 Love P E, Santoro S A. Antiphospholipid antibodies: anticardiolipin and the lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders: prevalence and clinical significance. Ann Intern Med . 1990; 112 682-698
- 49 Roberts W C, High S T. The heart in systemic lupus erythematosus. Curr Probl Cardiol . 1999; 24 1-56
- 50 Cheng T O. Pulmonary hypertension in systemic lupus erythematosus. Mayo Clin Proc . 1999; 74 845
- 51 Wilson L, Tomita T, Braniecki M. Fatal pulmonary hypertension in identical twins with systemic lupus erythematosus. Hum Pathol . 1991; 22 295-297
- 52 Evans J. Antinuclear antibody testing in systemic autoimmune disease. Clin Chest Med . 1998; 19 613-625
- 53 Burdt M, Hoffman R W, Deutscher S L, Wang G S, Johnson J C, Sharp G. Long-term outcome in mixed connective tissue disease. Arthr Rheum . 1999; 42 899-909
- 54 Sharp G C, Irvin W S, Tan E M, Gould R G, Holman R H. Mixed connective tissue disease: an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen. Am J Med . 1972; 52 148-159
- 55 Prakash U BS. Respiratory complications in mixed connective tissue disease. Clin Chest Med . 1999; 19 733-746
- 56 Wiener-Kronish J P, Soloinger A M, Warnock M L, Churg A, Ordonez N, Golden J A. Severe pulmonary involvement in mixed connective tissue disease. Am Rev Respir Dis . 1981; 124 499-503
- 57 Sullivan W D, Hurst D J, Harmon C E. et al . A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease. Medicine . 1984; 63 92-107
- 58 Fujii M, Adachi S, Shimizu T. Interstitial lung disease in rheumatoid arthritis: assessment with high-resolution computed tomography. J Thorac Imag . 1993; 8 54-62
- 59 Gabbay E, Tarala R, Will R. Interstitial lung disease in recent onset rheumatoid arthritis. Am J Respir Crit Care Med . 1997; 156 528-535
- 60 Remy-Jardin M, Remy J, Cortet B. Lung changes in rheumatoid arthritis: CT findings. Radiology . 1994; 193 375-382
- 61 Tanoue L T. Pulmonary manifestations of rheumatoid arthritis. Clin Chest Med . 1998; 19 667-685
- 62 Gardner D L, Duthie J R, MacLeod J, Allen W SH. Pulmonary hypertension in RA: report of a case study with intimal sclerosis of pulmonary and digital arteries. Scott Med J . 1957; 2 183-186
- 63 Helmers R, Galvin J, Hunninghake G W. Pulmonary manifestations associated with rheumatoid arthritis. Chest . 1991; 100 235-238
- 64 Dawson J K, Goodson N G, Graham D R, Lynch M P. Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology . 2000; 39 1320-1325
- 65 Okano Y, Steen V D, Medsger T A. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemc sclerosis. Arthritis Rheum . 1992; 35 95-100
- 66 Rich S, Levy P S. Characteristics of surviving and nonsurviving patients with primary pulmonary hypertension. Am J Med . 1984; 76 573-578
- 67 Fuster V, Steele P M, Edwards W D, Gersh B, McGoon M, Frye R L. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation . 1984; 70 580-587
- 68 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
- 69 Barst R J, Rubin L J, Long W A. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med . 1996; 334 296-302
- 70 McLaughlin V V, Genthner D E, Panella M M, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med . 1998; 338 273-277
- 71 McLaughlin V V, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation . 2002; 106 1477-1482
- 72 Badesch D B, Tapson V F, McGoon M D. et al . Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. Ann Intern Med . 2000; 132 425-434
- 73 Menon N, McAlpine L, Peacock A J, Madhok R. The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis. Arthr Rheum . 1998; 41 466-469
- 74 Klings E S, Hill N S, Leong M H, Simms R W, Korn J H, Farber H W. Systemic sclerosis-associated pulmonary hypertension. Arthr Rheum . 1999; 42 2638-2645
- 75 Strange C, Bolster M, Mazur J, Taylor M, Gossage J R, Silver R. Hemodynamic effects of epoprostenol in patients with systemic sclerosis and pulmonary hypertension. Chest . 2000; 118 1077-1082
- 76 Sitbon O, Humbert M, Sanchez O. Survival in pulmonary hypertension associated with connective tissue diseases (PH-CTD) treated with long-term epoprostenol (PGI2): comparison with primary pulmonary hypertension (PPH). Am J Respir Crit Care Med . 1999; 159 A158
- 77 Barst R J. Experience and reason. Chest . 2000; 117 2-5
- 78 Robbins I M, Gaine S P, Schilz R, Tapson V F, Rubin L J, Loyd J E. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest . 2000; 117 14-18
- 79 Horn E M, Barst R J, Poon M. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest . 2000; 118 1229-1230
- 80 Galie N, Grigioni F, Bacchi-Reggiani L. Relation of endothelin-1 to survival in patients with primary pulmonary hypertension. Eur J Clin Invest . 1996; 26 A48
- 81 Yamane K. Endothelin and collagen vascular disease: a review with special reference to Raynaud's phonomenon and systemic sclerosis. Intern Med . 1994; 33 579-582
- 82 Chatterjee K, De Marco T, Alpert J S. Pulmonary hypertension: hemodynamic diagnosis and management. Arch Intern Med . 2002; 162 1925-1933
- 83 Williamson D J, Wallman L L, Jones R. et al . Hemodynamic effects of bosentan, an endothelin receptor antagonist, in patients with pulmonary hypertension. Circulation . 2000; 102 411-418
- 84 Channick R N, Simonneau G, Sitbon O. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomized placebo controlled study. Lancet . 2001; 358 1119-1123
- 85 Rubin L J, Badesch D B, Barst R J. et al . Bosentan therapy for pulmonary arterial hypertension. N Engl J Med . 2002; 346 896-903
- 86 Lepore J J, Marco A, Pereira N L. et al . Effect of sildenafil on the acute pulmonary vasodilator response to inhaled nitric oxide in adults with primary pulmonary hypertension. Am J Cardiol . 2002; 90 677-680
- 87 Rosas I, Conte J, Yang S. Lung transplantation and systemic sclerosis. Ann Transplantation . 2000; 5 38-43
- 88 Arcasoy S M, Kotloff R M. Lung transplantation. N Engl J Med . 1999; 340 1081-1091