Clinical Reviews in Allergy & Immunology

, Volume 23, Issue 3, pp 247–261 | Cite as

Cardiac involvement in systemic autoimmune diseases

  • Piersandro Riboldi
  • Maria Gerosa
  • Cristina Luzzana
  • Luca Catelli


The heart and the vascular system are frequent and characteristic targets of several systemic autoimmune diseases, in particular Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA) and Systemic Sclerosis (SSc). In this chapter we review the classic cardiac abnormalities and the more recent data about cardiovascular involvement as part of a major disease complication determining a substantial morbidity and mortality.

In addition to the classic cardiac abnormalities involving the heart structures, acute and chronic ischemic heart disease and cerebrovascular accidents are threatening clinical manifestations of SLE and RA associated to an early accelerated atherosclerosis. Immune-mediated inflammation is now recognized as an important factor involved in the pathogenesis of atherosclerosis.

Ongoing clinical studies are being devised to find specific risk factors associated with systemic autoimmune diseases and/or treatment regimens. Hopefully, prophylactic measures should be available within the next few years.

Index Entries

Autoimmune diseases heart diseases Systemic Lupus Erythematosus scleroderma Rheumatoid Arthritis Sjögren Syndrome polymyositis 


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  1. 1.
    Libman, E. and Sacks, B. (1924), Arch. Intern. Med. 33, 701–737.Google Scholar
  2. 2.
    Harvey, A. M., Shulman, L. E., Tumulty, P. A., et al. (1954), Medicine 33, 291–437.PubMedCrossRefGoogle Scholar
  3. 3.
    Estes, D. and Christian, C. L. (1971), Medicine (Baltimore) 50, 85–95.Google Scholar
  4. 4.
    Grigor, R., Edmonds, J., Lewkonia, R., Bresnihan B., and Hughes, G. R. (1978). Ann. Rheum. Dis. 37, 121–128.PubMedGoogle Scholar
  5. 5.
    Bulkley, B. H. and Roberts, W. C. (1975), Am. J. Med. 58, 243–264.PubMedCrossRefGoogle Scholar
  6. 6.
    Urowitz, M. B., Bookman, A. A., Koehler, B. E., Gordon, D. A., Smythe, H. A., and Ogryzlo, M. A. (1976), Am. J. Med. 60, 221–225.PubMedCrossRefGoogle Scholar
  7. 7.
    Trager, J. and Ward, M. M. (2001), Curr. Opin. Rheumatol. 13, 345–351.PubMedCrossRefGoogle Scholar
  8. 8.
    Petri, M. (1998), Systemic lupus erythematosus and the cardiovascular system: the heart, in Systemic Lupus Erythematosus, Lahita, R. G., ed. New York, Churchill Livingstone.Google Scholar
  9. 9.
    Roldan, C. A., Shively, B. K., and Crawford, M. H. (1996), N. Engl. J. Med. 335, 1424–1430.PubMedCrossRefGoogle Scholar
  10. 10.
    Hojnik, M., George, J., Ziporen, L., and Shoenfeld Y. (1996), Circulation 93, 1579–1587.PubMedGoogle Scholar
  11. 11.
    Sasson, Z., Rasooly, Y., Chow, C. W., Marshall, S., and Urowitz, M. B. (1992), Am. J. Cardiol. 69, 1629–1634.PubMedCrossRefGoogle Scholar
  12. 12.
    Roman, M. J., Salmon, J. E., Sobel, R., Lokshin, M., Sammaritano, L., Schwartz, J. E., and Devereux, R. B. (2001), Am. J. Cardiol. 87, 663–666.PubMedCrossRefGoogle Scholar
  13. 13.
    Aranow, C. and Ginzler, E. M. (2000), Lupus 9, 166–169.PubMedCrossRefGoogle Scholar
  14. 14.
    Ward, M. (1999), Arthritis Rheum. 42, 338–346.PubMedCrossRefGoogle Scholar
  15. 15.
    Esdaile, J. M., Abrahamowicz, M., Grodzicky, T., Li, Y., Panaritis, C., du Berger, R., et al., (2001), Arthritis Rheum. 44, 2331–2337.PubMedCrossRefGoogle Scholar
  16. 16.
    Manzi, S., Selzer, F., Sutton-Tyrrell, K., Fitzgerald, S. G., Rairie, J. E., Tracy, R. P., and Kuller, L. H. (1999), Arthritis Rheum. 42, 51–60.PubMedCrossRefGoogle Scholar
  17. 17.
    O'Leary, D. H., Polak, J. F., Kronmal, R. A., Manolio, T. A., Burke, G. L., and Wolfson, S. K. (1999), N. Engl. J. Med. 340, 14–22.PubMedCrossRefGoogle Scholar
  18. 18.
    Petri, M. (2000), Lupus 9, 170–175.PubMedCrossRefGoogle Scholar
  19. 19.
    Lockshin, M. D., Salmon, J. E., and Roman, M. J. (2001), Arthritis Rheum. 44, 2215–2217.PubMedCrossRefGoogle Scholar
  20. 20.
    Ross, R. (1999), N. Engl. J. Med. 340, 115–126.PubMedCrossRefGoogle Scholar
  21. 21.
    George, J., Haratz, D., and Shoenfeld, Y. (2001), Rheum. Dis. Clin. North Am. 27, 603–610.PubMedCrossRefGoogle Scholar
  22. 22.
    Petri, M., Roubenoff, R., Dallal, G. E., Nadeau, M. R., Selhub, J., and Rosenberg, I. H. (1996), Lancet 348, 1120–1124.PubMedCrossRefGoogle Scholar
  23. 23.
    Falaschi, F., Rayelli, A., Martignoni, A., Migliavacca, D., Sartori, M., Pistorio, A., et al. (2001), Arthritis Rheum. 43, 1405–1409.CrossRefGoogle Scholar
  24. 24.
    Moroni, G., La Marchesina, U., Banfi, G., Nador, F., Viganò, E., Marconi, M., et al. (1995), Clin. Nephrology 43, 20–28.Google Scholar
  25. 25.
    Tseng, C. E. and Buyon, J. P. (1997), Rheum. Dis. Clin. North Am. 23, 31–54.PubMedCrossRefGoogle Scholar
  26. 26.
    Sharp, G. S., Irvin, W. S., Tan, E. M., Gould, R. G., and Holman, H. R. (1972), Am. J. Med. 52, 148–159.PubMedCrossRefGoogle Scholar
  27. 27.
    Alpert, M. A., Goldberg, S. H., Singsen, B. H., Durham, J. B., Sharp, G. C., Ahmad, M., et al. (1983), Circulation 68, 1182–1193.PubMedGoogle Scholar
  28. 28.
    Oetgen, W. J., Mutter, M. L., Davia, J. E., and Laeless, O. J. (1983), Chest 83, 185–188.PubMedGoogle Scholar
  29. 29.
    Comens, S. M., Alpert, M. A., Sharp, G. C., Pressly, T. A., Kelly, D. L., Hazelwood, S. E., and Mukerji, V. (1989), Am. J. Cardiol. 63, 369–370.PubMedCrossRefGoogle Scholar
  30. 30.
    Leung, W. H., Wong, K. L., Lau, C. P., Wong, C. K., Cheng, C. H., and Tai, Y. T. (1990), J. Rheumatol. 17, 485–488.PubMedGoogle Scholar
  31. 31.
    Weiner-Kronish, J. P., Solinger, A. M., Warnock, M. S., Churg, A., Ordonez, N., and Golden, J. A. (1981), Am. Rev. Respir. Dis. 124, 499–503.Google Scholar
  32. 32.
    Harmon, C., Wolfe, J. F., Lillard, S., Held, C., Coedon, R., and Sharp, G. C. (1976), Arthritis Rheum. 19, 801–807.Google Scholar
  33. 33.
    Hosoda, Y., Suzuki, Y., Takano, M., Tojo, T., and Homma, M. (1987), J. Rheumatol. 14, 826–830.PubMedGoogle Scholar
  34. 34.
    Leung, W. H., Wong, K. L., Lau, C. P., Wong, C. K., Cheng, C. H., and Tai, Y. T. (1990), Cardiology 77, 93–100.PubMedGoogle Scholar
  35. 35.
    Lash, A. D., Wittman, A. L., and Quismorio, F. P. (1986), Semin. Arthritis Rheum. 15, 288–296.PubMedCrossRefGoogle Scholar
  36. 36.
    Hammann, C., Genton, C. Y., Delabays, A., Bischoff Delaloye, A., Bogousslavsky, J., and Spertini, F. (1999), Clin. Rheumatol. 18 85–87.PubMedCrossRefGoogle Scholar
  37. 37.
    Golan, T. D., Keren, D., Elias, N., Naschitz, J. E., Toubi, E., Misselevich, I., and Yeshurun, D., (1997), Lupus 6, 505–508.PubMedGoogle Scholar
  38. 38.
    Levin, M. D., Zoet-Nugteren, S. K., Markusse, H. M. (1999), Lancet 354, 128–129.PubMedCrossRefGoogle Scholar
  39. 39.
    Yoshioka, K., Tegoschi, H., Yoshida, T., Uoshima, N., and Kasamatsu, Y. (1999), Lancet 354, 1996.PubMedCrossRefGoogle Scholar
  40. 40.
    Gyöngyösi, M., Pokorny, G., Jambrik, Z., Kovacs, L., Kovacs, A., Makula, E., and Csanady, M. (1996), Ann. Rheum. Dis. 55, 450–454.PubMedGoogle Scholar
  41. 41.
    Rantapää-Dahlqvist, S., Backman, C., Sandgren, H., and Ostberg, Y. (1993), Clin. Rheumatol. 12, 214–218.PubMedCrossRefGoogle Scholar
  42. 42.
    Janosik, D. L., Osborn, T. G., Moore, T. L., Shah, D. G., and Kenney, R. G. (1989) Semin. Arthritis. Rheum. 19, 191–200.PubMedCrossRefGoogle Scholar
  43. 43.
    Harvey, G. R., Butts, S., Rands, A. L., Patel, Y., and McHugh, N. J. (1999), Clin. Exp. Immunol. 117, 395–402.PubMedCrossRefGoogle Scholar
  44. 44.
    McWhorter, J. E. and LeRoy, E. C. (1974), Am. J. Med. 57, 566–575.PubMedCrossRefGoogle Scholar
  45. 45.
    D'Angelo, W. A., Fries, J. F., Masi, A. T., and Shulman, L. E. (1969), Am. J. Med. 46, 428–440.PubMedCrossRefGoogle Scholar
  46. 46.
    Thompson, A. E. and Pope, J. E. (1998), Br. J. Rheumatol. 37, 1320–1323.PubMedCrossRefGoogle Scholar
  47. 47.
    Gowda, R. M., Khan, I. A., Sacchi, T. J., and Vasavada, B. C. (2001), Angiology 52, 59–62.PubMedGoogle Scholar
  48. 48.
    Follansbee, W. P., Miller, T. R., Curtiss, E. I., Orie, J. E., Bernstein, R. L., Kiernan, J. M., Medsger, T. A., Jr. (1990), J. Rheumatol. 17, 656–662.PubMedGoogle Scholar
  49. 49.
    Coghlan, J. G. and Mukerjee, D. (2001), Curr. Opin. Rheumatol. 13, 495–499.PubMedCrossRefGoogle Scholar
  50. 50.
    Kahan, A., Devaux, J. Y., Amor, B., Menkes, C. J., Weber, S., Nitenberg, A., et al. (1986), N. Engl. J. Med. 314, 1397–1402.PubMedCrossRefGoogle Scholar
  51. 51.
    Kahan, A., Devaux, J. Y., Amor, B., Menkes, C. J., Weber, S., Foult, J. M., et al. (1986), Ann. Rheum. Dis. 45, 718–725.PubMedGoogle Scholar
  52. 52.
    Kahan, A., Devaux, J. Y., Amor, B., Menkes, C. J., Weber, S., Venot, A., et al. (1988), J. Rheumatol. 15 1395–1400.PubMedGoogle Scholar
  53. 53.
    Pace, L., Capelli, L., Bove, E., Coppola, N., Ciarmiello, A., Sorrentino, S., and Salvatore, M. (1992), J. Nucl. Med. 33, 68–72.PubMedGoogle Scholar
  54. 54.
    Siegel, R. J., O'Connor, B., Mena, I., and Criley, J. M. (1984), Am. Heart J. 108, 1469–1476.PubMedCrossRefGoogle Scholar
  55. 55.
    Handa, R., Gupta, K., Malthortra, A., Jain, P., Kamath, P. K., Agarwal, P., et al. (1999), Clin. Rheumatol. 18, 136–139.PubMedCrossRefGoogle Scholar
  56. 56.
    Kazzam, E., Waldenstrom, A., Landelius, J., Hallgren, R., Arvidsson, A., and Caidhal, K. (1990), J. Intern. Med. 228, 183–192.PubMedCrossRefGoogle Scholar
  57. 57.
    Murata, I., Takenaka, K., Shinohara, S., Suzuki, T., Sasaki, T., and Yamamoto, K. (1998), Am. Heart J. 135, 960–969.PubMedCrossRefGoogle Scholar
  58. 58.
    Ferri, C., Bernini, L., Bongiorni, M. G., Levorato, D., Viegi, G., Bravi, P., et al. (1985), Arthritis Rheum. 28, 1259–1266.PubMedCrossRefGoogle Scholar
  59. 59.
    Clements, P. J., Furst, D. E., Cabeen, W., Tashkin, D., Paulus, H. E., and Roberts, N. (1981), Am. J. Med. 71, 38–46.PubMedCrossRefGoogle Scholar
  60. 60.
    Follansbee, W. P., Curtiss, E. I., Rahko, P. S., Medsger, T. A., Jr., Lavine, S. J., Owens, G. A., and Steen, V. D. (1985), Am. J. Med. 79, 183–192.PubMedCrossRefGoogle Scholar
  61. 61.
    Deswal, A. and Follansbee, W. P. (1996), Rheum. Dis. Clin. North Am. 22, 841–860.PubMedCrossRefGoogle Scholar
  62. 62.
    Lebowitz, W. B. (1963), Ann. Intern. Med. 58, 102–123.PubMedGoogle Scholar
  63. 63.
    Nomeir, A. M., Turner, R., Watts, E., Smith, D., West, G., and Edmonds, J. (1973), Ann. Intern. Med. 79, 800–806.PubMedGoogle Scholar
  64. 64.
    Mitchell, D. M., Spitz, P. W., Young, D. Y., Bloch, D. A., MsShane, D. J., and Fries, J. F. (1986), Arthritis Rheum. 29, 706–714.PubMedCrossRefGoogle Scholar
  65. 65.
    Wolfe, F., Mitchell, D. M., Sibley, J. T., Fries, J. F., Bloch, D. A., Williams, C. A., et al. (1994), Arthritis Rheum. 37, 481–494.PubMedCrossRefGoogle Scholar
  66. 66.
    Myllykangans-Luosujärvi, R., Aho, K., Kautiainen, H., and Isomäki, H. (1995), J. Rheumatol. 22, 1065–1067.Google Scholar
  67. 67.
    Wällberg-Jonsson, S., Öhman, M.-L., and Dahlqvist, S. R. (1997), J. Rheumatol. 24, 445–451.PubMedGoogle Scholar
  68. 68.
    Kirk, J. and Cosh, J. (1969), Q. J. Med. 38, 397–423.PubMedGoogle Scholar
  69. 69.
    Wilkinson, M. (1962), BMJ 2, 1723.PubMedGoogle Scholar
  70. 70.
    Hara, K. S., Ballard, D. J., Ilstrup, D. M., Connolly, D. C., and Vollertsen, R. S. (1990), Medicine (Baltimore) 69, 81–91.Google Scholar
  71. 71.
    Escalante, A., Kaufman, R. L., Quismorio, F. P., Jr., and Beadmore, T. D. (1990), Semin. Arthritis Rheum. 20, 148–163.PubMedCrossRefGoogle Scholar
  72. 72.
    Thadani, V., Iveson, J. M. I., and Wright, V. (1975), Medicine (Baltimore) 54, 261–270.CrossRefGoogle Scholar
  73. 73.
    Guédes, C., Bianchi-Fior, P., Cormier, B., Barthelemy, B., Rat, A. C., and Boissier, M. C. (2001), Arthritis Care Res. 45, 129–135.CrossRefGoogle Scholar
  74. 74.
    Corrao, S., Salli, L., Arnone, S., Scaglione, R., Amato, V., Cecala, M., et al. (1995), Euro. Heart J. 16, 253–256.Google Scholar
  75. 75.
    Wislowska, M., Sypula, S., and Kowalic, I. (1998), Clin. Rheumatol. 17, 369–377.PubMedCrossRefGoogle Scholar
  76. 76.
    Mody, G. M., Stevens, J. E., and Meyers, O. L. (1987), Q. J. Med. 65, 921–928.PubMedGoogle Scholar
  77. 77.
    Maione, S., Valentini, G., Giunta, A., Tirri, R., Giacummo, A., Lippolis, C., et al. (1993), Cardiology 83, 234–239.PubMedCrossRefGoogle Scholar
  78. 78.
    Prakash, R., Atassi, A., Poske, R., and Rosen, K. (1973), N. Engl. J. Med. 289 597–600.PubMedCrossRefGoogle Scholar
  79. 79.
    Gordon, D. A., Stein, G. L., and Broder, I. (1973), Am. J. Med. 54, 445–452.PubMedCrossRefGoogle Scholar
  80. 80.
    Hardouin, P., Thevenon, A., Beuscart, R., Tison-Muchery, F., Duquesnoy, B., Thery, C., et al. (1988), Rev. Rheum. Mal. Osteoartic 55, 683–687.Google Scholar
  81. 81.
    Ahern, M., Lever, J. V., and Cash, J. (1983), Ann. Rheum. Dis. 42, 389–397.PubMedCrossRefGoogle Scholar
  82. 82.
    Wislowska, M., Sypula, S., and Kowalik, I. (1999), Rheumatol. Int. 18, 163–169.PubMedCrossRefGoogle Scholar
  83. 83.
    Stern, R., Goodbold, J. H., Chess, Q., and Kagen, L. J. (1984), Arch. Intern. Med. 44, 2185–2189.CrossRefGoogle Scholar
  84. 84.
    Lie, J. T. (1995), J. Rheumatol. 22, 809–811.PubMedGoogle Scholar
  85. 85.
    Spiera, R. and Kagen, L. (1998), Curr. Opin. Rheumatol. 10, 556–561.PubMedCrossRefGoogle Scholar
  86. 86.
    Gonzales-Lopez, L., Gamez-Nava, J. I., Sanchez, L., Rosas, E., Suarez-Almaroz, M., Cardona-Munoz, C., and Ramos-Remus, C. (1996), Clin. Exp. Rheumatol. 14, 373–379.Google Scholar

Copyright information

© Humana Press Inc. 2002

Authors and Affiliations

  • Piersandro Riboldi
    • 1
    • 2
  • Maria Gerosa
    • 1
    • 2
  • Cristina Luzzana
    • 1
    • 2
  • Luca Catelli
    • 1
    • 2
  1. 1.Department of Internal MedicineUniversity of MilanMilanItaly
  2. 2.Allergy and Clinical Immunology UnitIRCCS Istituto AuxologicoMilanItaly

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