Skip to main content
SpringerLink
Log in

Pulmonary hypertension in collagen vascular disease

  • Original Articles
  • Published:
Comprehensive Therapy

Summary

Infrequent in occurrence, insidious in onset, and often relentless in its course, the pulmonary hypertension associated with collagen vascular disease remains a challenging syndrome to diagnose and treat. The subtlety and lack of specificity of its early symptoms and signs, the lack of availability of sensitive, noninvasive, accurate diagnostic tests, and the limited data from large-scale therapeutic trials in this population all pose many frustrations for patient and physician. Supportive, symptomatic therapy remains the mainstay of treatment. The results of recently completed and ongoing clinical trials investigating the therapeutic role of newer vasodilators suggest that for patients with relatively isolated pulmonary arteriopathy, additional therapeutic options will soon be available.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Asherson RA, Oakley M. Pulmonary hypertension and systemic lupus erythematosus (editorial).J Rheumatol. 1986;13:1–5.

    PubMed  CAS  Google Scholar 

  2. Bunch TW, Tancredi RG, Lie JT. Pulmonary hypertension in polymyositis.Chest. 1981;79:105–107.

    Article  PubMed  CAS  Google Scholar 

  3. Kobayashi H, Sano T, Fi K, et al. Mixed connective tissue disease with fatal pulmonary hypertension.Acta Pathologica Japonicum. 1982;32:1121–1129.

    CAS  Google Scholar 

  4. Stupi AM, Steen VD, Owens GR, et al. Pulmonary hypertension in CREST syndrome variant of systemic sclerosis (scleroderma).Arthritis Rheum. 1986;29:515–524.

    Article  PubMed  CAS  Google Scholar 

  5. Fahey PJ, Utell MJ, Condemi JJ, et al. Raynaud’s phenomenon of the lung.Am J Med. 1984;76:263–269.

    Article  PubMed  CAS  Google Scholar 

  6. Farb A, Burke AP, Virmani R. Pulmonary hypertension caused by chronic left heart failure, obstruction of pulmonary venous return and parenchymal lung disease. In: Saldana MJ, ed.Pathology of Pulmonary Disease. Philadelphia, Pa: JB Lippincott; 1994.

    Google Scholar 

  7. Lie JT. Pulmonary involvement in collagen vascular disorders. In: Saldana MJ, ed.Pathology of Pulmonary Disease. Philadelphia, Pa: JB Lippincott; 1994:781–790.

    Google Scholar 

  8. Koh ET, Lee P, Gladman DO, Abu-Shakra M. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients.Br J Rheum. 1996;35:989–993.

    Article  CAS  Google Scholar 

  9. Battle RW, Davitt MA, Cooper Sm, et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma.Chest. 1996;110:1515–1519.

    Article  PubMed  CAS  Google Scholar 

  10. Ungerer RG, Tashkin DP, Furst D, et al. Prevalence and clinical correlates of pulmonary arterial hypertension in progressive systemic sclerosis.Am J Med. 1983;75:65–74.

    Article  PubMed  CAS  Google Scholar 

  11. Salerni R, Rodnan GP, Leon DF, et al. Pulmonary hypertension in the CREST syndrome variant of progressive systemic sclerosis (scleroderma).Ann Intern Med. 1977;86:394–399.

    PubMed  CAS  Google Scholar 

  12. Yousem SA. The pulmonary pathologic manifestations of the CREST syndrome.Hum Pathol. 1990;21:467–474.

    Article  PubMed  CAS  Google Scholar 

  13. Prakesh UBS. Rheumatological diseases. In: Murray JF, ed.Pulmonary Complications of Systemic Disease. New York, NY: Marcel-Dekker; 1992.

    Google Scholar 

  14. Ohtsuka T, Hasegawa A, Nakano A, et al. Nailfold capillary abnormality and pulmonary hypertension in systemic sclerosis.Int J Derm. 1997;36:116–122.

    Article  PubMed  CAS  Google Scholar 

  15. Murata I, Takenaka K, Yoshinoya S, et al. Clinical evaluation of pulmonary hypertension in systemic sclerosis and related disorders: a Doppler echocardiographic study of 135 Japanese patients.Chest. 1997;111:36–43.

    Article  PubMed  CAS  Google Scholar 

  16. Denton CP, Cailes JB, Phillips GD, et al. Comparison of Doppler echocardiography and right heart catheterization to assess pulmonary hypertension in systemic sclerosis.Br J Rheum. 1997;36:239–243.

    Article  CAS  Google Scholar 

  17. Quismorio FP, Sharma O, Koss M, et al. Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus.Semin Arthritis Rheum. 1984;13:349–359.

    Article  PubMed  Google Scholar 

  18. Asherson RA, Hackett D, Gharaci AE, et al. Pulmonary hypertension and systemic lupus erythematosus: a report of three cases.J Rheumatol. 1986;13:416–420.

    PubMed  CAS  Google Scholar 

  19. Orens JB, Martinez FJ, Lynch JP. Pleuropulmonary manifestations of systemic lupus erythematosus.Rheum Dis Clin North Am. 1994;20:159–193.

    PubMed  CAS  Google Scholar 

  20. Fayemi AO. The lung in systemic lupus erythematosus: a clinicopathologic study of 20 cases.Mt Sinai J Med. 1975;42:110–118.

    PubMed  CAS  Google Scholar 

  21. Fayemi AO. Pulmonary vascular disease in systemic lupus erythematosus.Am J Clin Pathol. 1976;65:284–290.

    PubMed  CAS  Google Scholar 

  22. Wiedemann HP, Matthay RA. Pulmonary manifestations of the collagen vascular diseases.Clin Chest Med. 1989;10:677–722.

    PubMed  CAS  Google Scholar 

  23. Haupt HM, Moore GW, Hutchins GM. The lung in systemic lupus erythematosus: analysis of the pathologic changes in 120 patients.Am J Med. 1981;71:791–798.

    Article  PubMed  CAS  Google Scholar 

  24. Mahowald ML, Weir EK, Ridley DJ, et al. Pulmonary hypertension in systemic lupus erythematosus: effect of vasodilators on pulmonary hemodynamics.J Rheumatol. 1985;12:773–777.

    PubMed  CAS  Google Scholar 

  25. Scherak O, Kolarz G. Systemic lupus erythematosus and pulmonary hypertension.J Rheumatol. 1981;40:131–134.

    CAS  Google Scholar 

  26. Marchesoni A, Messina K, Carrieri P, et al. Pulmonary hypertension and systemic lupus erythematosus.Clin Exp Rheumatol. 1983;1:247–250.

    PubMed  CAS  Google Scholar 

  27. Schwartzberg M, Lieverman DH, Getzoff B, et al. Systemic lupus erythematosus and pulmonary vascular hypertension.Arch Intern Med. 1984;144:605–607.

    Article  PubMed  CAS  Google Scholar 

  28. Wiener-Kronish JP, Solinger AM, Warnock ML, et al. Severe pulmonary involvement in mixed connective tissue disease.Am Rev Respir Dis. 1981;124:499–503.

    PubMed  CAS  Google Scholar 

  29. Alpert MA, Goldberg SH, Singsen BH, et al. Mixed connective tissue disease with fatal pulmonary hypertension in adults.Circulation. 1983;68:1182–1193.

    PubMed  CAS  Google Scholar 

  30. Ueda N, Mimura K, Maeda H, et al. Mixed connective tissue disease with fatal pulmonary hypertension and a review of the literature.Virchows Arch. 1984;404:335–340.

    Article  CAS  Google Scholar 

  31. Debow CE, Lie JT, Tancredi RG, et al. Cardiac involvement in polymyositis: clinicopathologic study of 20 autopsied patients.Arthritis Rheum. 1979;22:1088–1092.

    Article  Google Scholar 

  32. Caldwell IW, Aitchison JD. Pulmonary hypertension in dermatomyositis.Br Heart J. 1956;18:273–276.

    Article  PubMed  CAS  Google Scholar 

  33. Helmers R, Galvin J, Hunninghake GW. Pulmonary manifestations associated with rheumatoid arthritis.Chest. 1991;100:235–238.

    Article  PubMed  CAS  Google Scholar 

  34. Kay JM, Banik S. Unexplained pulmonary hypertension with pulmonary arteritis in rheumatoid disease.Br J Med. 1977;71:53–59.

    CAS  Google Scholar 

  35. Turner-Warwick M, Evans RC. Pulmonary manifestations of rheumatoid disease.Clin Rheum Dis. 1977;3:549–564.

    Google Scholar 

  36. Yoshio T, Masuyama J, Sumiya M, et al. Antiendothelial cell antibodies and their relations to pulmonary hypertension in systemic lupus erythematosus.J Rheumatol. 1994;21:2058–2063.

    PubMed  CAS  Google Scholar 

  37. Palevsky HI. Pulmonary hypertension and right-sided heart failure. In: Carlson RW, Geheb MA, eds.The Principles and Practice of Medical Intensive Care. Philadelphia, Pa: WB Saunders; 1993:838–849.

    Google Scholar 

  38. Rosen S, Johanson K, Lindberg K, et al. Multicenter evaluation of a kit for activated protein C resistance on various coagulation instruments using plasmas from healthy individuals.Thromb Haemost. 1994;72:255–260.

    PubMed  CAS  Google Scholar 

  39. Fuster V. Steele PM, Edwards WD, et al. Primary pulmonary hypertension: natural history and the importance of thrombosis.Circulation. 1984;70:580–587.

    PubMed  CAS  Google Scholar 

  40. Rich S, Kieras K, Hart K, et al. Antinuclear antibodies in primary pulmonary hypertension.J Am Coll Cardiol. 1986;8:1307–1311.

    PubMed  CAS  Google Scholar 

  41. Okano Y, Steen VD, Medsger TA. Autoantibody to U3 nucleolar ribonucleoprotein (fibrallarin) in patients with systemic sclerosis.Arthritis Rheum. 1992;35:95–100.

    Article  PubMed  CAS  Google Scholar 

  42. Hoffman T, Kaspers W, Meinertz T, et al. Echocardiographic evaluation of patients with clinically suspected arterial emboli.Lancet. 1990;336:1421–1424.

    Article  Google Scholar 

  43. Pearson AC, Labovitz AJ, Tatineni S, et al. Superiority of transesophageal echocardiography in detecting cardiac source of embolism in patients with cerebral ischemia of uncertain etiology.J Am Coll Cardiol. 1991;17:66–72.

    PubMed  CAS  Google Scholar 

  44. Kelley MA, Carson JL, Palevsky HI, et al. Diagnosing pulmonary embolism: new facts and strategies.Ann Intern Med. 1991;114:300–306.

    PubMed  CAS  Google Scholar 

  45. Palevsky HI, Edmunds LH. Pulmonary thromboembolism. In: Edmunds LH, ed.Cardiac Surgery in the Adult. New York, NY: McGraw Hill; 1997:1319–1344.

    Google Scholar 

  46. Gefter WB, Hatabu H, Holland GA, et al. Pulmonary thromboembolism: recent developments in diagnosis with CT and MR imaging.Radiology. 1995;197:561–574.

    PubMed  CAS  Google Scholar 

  47. Rubin LJ. ACCP Consensus Statement. Primary pulmonary hypertension.Chest. 1993;104:236–250.

    Article  PubMed  CAS  Google Scholar 

  48. Rawson AJ, Woske HM. A study of etiologic factors in so-called primary pulmonary hypertension.Arch Intern Med. 1960;105:133–243.

    Google Scholar 

  49. Ferguson GT, Cherniak RM. Management of chronic obstructive pulmonary disease.N Engl J Med. 1993;328:1017–1022.

    Article  PubMed  CAS  Google Scholar 

  50. Gaine SP, Rubin LJ. Medical and surgical treatment options for pulmonary hypertension.Am J Med Sci. 1998;315:179–184.

    Article  PubMed  CAS  Google Scholar 

  51. Flenley DC. Long-term home oxygen therapy.Chest. 1985;89:99–103.

    Article  Google Scholar 

  52. Rich S, Seidlitz M, Dodin E, et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension.Chest. 1998;114:787–792.

    Article  PubMed  CAS  Google Scholar 

  53. Palevsky HI, Fishman AP. Cor pulmonale: etiology and management.JAMA. 1990;263:2347–2353.

    Article  PubMed  CAS  Google Scholar 

  54. 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.

    PubMed  CAS  Google Scholar 

  55. Weir EK, Rubin LJ, Ayres SM, et al. The acute administration of vasodilators in primary pulmonary hypertension.Am Rev Respir Dis. 1989;140:1623–1630.

    PubMed  CAS  Google Scholar 

  56. Palevsky HI, Fishman AP. The management of primary pulmonary hypertension.JAMA. 1991;265:1014–1020.

    Article  PubMed  CAS  Google Scholar 

  57. Galie N, Ussia G. Passarelli P, et al. Role of pharmacologic tests in the treatment of primary pulmonary hypertension.Am J Cardiol. 1995;75:55A-62A.

    Article  PubMed  CAS  Google Scholar 

  58. Palevsky HI. Treatment of pulmonary hypertension. In: Leff AR, ed.Pulmonary and Critical Care Pharmacology and Therapeutics. New York, NY: McGraw-Hill; 1996:1099–1109.

    Google Scholar 

  59. Reeves JT, Groves BM, Turkevich D. The case for treatment of selected patients with primary pulmonary hypertension.Am Rev Respir Dis. 1986;134:342–346.

    PubMed  CAS  Google Scholar 

  60. Rich S, Kaufmann E, Levy PS. The effect of high does of calcium-channel blockers on survival in primary pulmonary hypertension.N Engl J Med. 1992;327:76–81.

    PubMed  CAS  Google Scholar 

  61. Cremona G, Higenbottam T. Role of prostacyclin in the treatment of primary pulmonary hypertension.Am J Cardiol. 1995;75:67A-71A.

    Article  PubMed  CAS  Google Scholar 

  62. Higenbottam TW, Butt AY, Dinh-Xaun AT, et al. Treatment of pulmonary hypertension with the continuous infusion of a prostacyclin analogue, iloprost.Heart. 1998;79:175–179.

    PubMed  CAS  Google Scholar 

  63. Palevsky HI, Long W, Crow J, et al. Prostacyclin and acetylcholine as screening agents for acute pulmonary vasodilator responsiveness in primary pulmonary hypertension.Circulation. 1990;82:2018–2026.

    PubMed  CAS  Google Scholar 

  64. Nootens M, Schrader B, Kaumann, E, et al. Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension.Chest. 1995;107:54057.

    Article  Google Scholar 

  65. Pepke-Zaba J, Higenbottam TW, Tuan Dinh-Xuan A, et al. Inhaled nitric oxide as a cause of selective vasodilation in pulmonary hypertension.Lancet. 1991;338:1173–1174.

    Article  PubMed  CAS  Google Scholar 

  66. Dantzker DR, D’Alonzo GE, Gianotti L, et al. Vasodilators and primary pulmonary hypertension: variability of long-term response.Chest. 1989;95:1185–1189.

    Article  PubMed  CAS  Google Scholar 

  67. Palevsky HI, Schloo BL, Pietra GG, et al. Primary pulmonary hypertension: vascular structure, morphometry, and responsiveness to vasodilator agents.Circulation. 1989;80:1207–1212.

    PubMed  CAS  Google Scholar 

  68. Niarchos AP, Whitman HH, Goldstein JE, et al. Hemodynamic effects of captopril in pulmonary hypertension.Ann Intern Med. 1987;107:565–568.

    Google Scholar 

  69. Alpert MA, Presley TA, Mukerji V, et al. Acute and long-term effects of nifedipine on pulmonary and systemic hemodynamics in patients with pulmonary hypertension associated with diffuse systemic sclerosis, the CREST syndrome and mixed connective tissue disease.Am J Cardiol. 1991;68:1689–1691.

    Google Scholar 

  70. Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy.Circulation. 1987;76:135–141.

    PubMed  CAS  Google Scholar 

  71. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension: results from a National Prospective Registry.Ann Intern Med. 1991;115:343–349.

    PubMed  CAS  Google Scholar 

  72. Inbar S, Schrader BJ, Kaufmann E, et al. Autoantibodies in patients with primary pulmonary hypertension.J Am Coll Cardiol. 1993;21:413–418.

    Article  PubMed  CAS  Google Scholar 

  73. Humbert M, Brenot F, Jagot J-L, et al. Treatment of severe pulmonary hypertension secondary to connective tissue diseases with continuous intravenous prostacyclin.Am J Respir Crit Care Med. 1997;155(4 suppl):A443.

    Google Scholar 

  74. Menon N, McAlpine L, Peacock AJ, Madhok R. The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis.Arthritis Rheum. 1998;41:466–469.

    Article  PubMed  CAS  Google Scholar 

  75. Badesch DB, Brundage BH, McGoon MD, et al. Continuous intravenous epoprostenol versus conventional therapy in pulmonary hypertension due to the scleroderma spectrum of disease: a 12-week, multicenter, randomized controlled trial.Circulation. 1998;98(suppl 1):S614.

    Google Scholar 

  76. Conte JV, Gaine SP, Orens JB, et al. The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation.J Heart Lung Trans. 1998, 17: 679–685.

    CAS  Google Scholar 

  77. Rubin LJ, Mondoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol): results of a randomized trial.Ann Intern Med. 1991;112:485–491.

    Google Scholar 

  78. Barst RJ, Rubin LJ, McGoon MD, et al. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin.Ann Intern Med. 1994;121:409–415.

    PubMed  CAS  Google Scholar 

  79. 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;334:296–301.

    Article  PubMed  CAS  Google Scholar 

  80. de la Mata J, Gomez-Sanchez MA, Aranzana M, et al. Long-term iloprost infusion therapy for severe pulmonary hypertension in patients with connective tissue diseases.Arthritis Rheum. 1994;37:1528–1533.

    Article  PubMed  Google Scholar 

  81. Torley HI, Madhok R, Capell HA, et al. A double-blind, randomized, multicentre comparison of two doses of intravenous iloprost in the treatment of Raynaud’s phenomenon secondary to connective tissue diseases.Ann Rheum Dis. 1991;50:800–804.

    Article  PubMed  CAS  Google Scholar 

  82. Belch JJF, Capel HA, Cooke Ed, et al. Oral iloprost as a treatment for Raynaud’s syndrome: a double-blind multicentre placebo-controlled study.Ann Rheum Dis. 1995;54:197–200.

    PubMed  CAS  Google Scholar 

  83. Olschewski H, Walmrath D, Schermuly R, et al. Aerosolized prostacyclin and iloprost in severe pulmonary hypertension.Ann Intern Med. 1996;124:820–824.

    PubMed  CAS  Google Scholar 

  84. Saji T, Ozawa Y, Ishikita T, et al. Short-term hemodynamic effect of a new oral PGI2 analogue, beraprost in primary and secondary pulmonary hypertension.Am J Cardiol. 1996;78:244–247.

    PubMed  CAS  Google Scholar 

  85. Rich S. Medical treatment of primary pulmonary hypertension: a bridge to transplantation?Am J Cardiol. 1995;75:63A-66A.

    Article  PubMed  CAS  Google Scholar 

  86. de Hoyos AL, Patterson GA, Maurer JR, et al. Pulmonary transplantation: early and late results: the Toronto Lung Transplant Group.J Thorac Cardiovasc Surg. 1992;103:295–306.

    PubMed  Google Scholar 

  87. Piqula FA, Griffith BP, Zenati MA, et al. Lung transplantation for respiratory failure resulting from systemic disease.Ann Thoracic Surg. 1997;64:1630–1634.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, University of Pennsylvania Health System, Philadelphia, PA

    Harold I. Palevsky MD & Indira Gurughagavatula MD

Authors
  1. Harold I. Palevsky MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Indira Gurughagavatula MD
    View author publications

    You can also search for this author in PubMed Google Scholar

About this article

Cite this article

Palevsky, H.I., Gurughagavatula, I. Pulmonary hypertension in collagen vascular disease. Compr Ther 25, 133–143 (1999). https://doi.org/10.1007/BF02889609

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02889609

Keywords

Search

Navigation