Pathophysiology and Behavioral Treatment of Scleroderma

  • Robert Freedman
  • Paul Wenig
Part of the NATO Conference Series book series (NATOCS, volume 19)


Scleroderma (progressive systemic sclerosis or PSS) is a connective tissue disorder distinguished by symmetric thickening of the skin, by abnormalities of the microvasculature and large blood vessels, and by degenerative fibrotic changes in the viscera, muscles and joints (Rodnan, 1979a). Although the etiology of the disease is unknown, mechanisms involving the vascular, immune, and connective tissue systems have been proposed (LeRoy, 1981). The vasospastic attacks of Raynaud’s phenomenon, consisting of pallor, cyanosis, and rubor in the fingers and sometimes the toes, occur in over 95% of patients with progressive systemic sclerosis (Rodnan, 1979a).


Systemic Sclerosis Capillary Blood Flow Peripheral Blood Flow Progressive Systemic Sclerosis Digital Artery 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Adair, J. & Theobald, D. Raynaud’s phenomenon: treatment of a severe case with biofeedback. Journal of the Indiana State Medical Association, 1978, 71, 990–993.Google Scholar
  2. American Rheumatism Association. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Bulletin on the Rheumatic Diseases, 1981, 31, (1), 1–6.Google Scholar
  3. Bulkley, B., Ridolfi, R., Salyer, W., & Hutchins, G. Myocardial lesions of progressive systemic sclerosis, a cause of cardiac dysfunction. Circulation, 1976, 53, 483–490.Google Scholar
  4. Campbell, P, & LeRoy, E. Pathogenesis of systemic sclerosis: a vascular hypothesis. Seminars in Arthritis and Rheumatism, 1975, 4, 351–368.CrossRefGoogle Scholar
  5. Cannon, P., Hassar, M., Case, D., Casarella, W., Sommers, S., & LeRoy, E. The relationship of hypertension and renal failure in scleroderma to structural and functional abnormalities of the renal cortical circulation. Medicine, 1974, 53, 1–46.CrossRefGoogle Scholar
  6. Coffman, J. Skin blood flow in scleroderma. Journal of Laboratory and Clinical Medicine, 1970, 76, 480–484.Google Scholar
  7. Coffman, J., & Cohen, A. Total and capillary fingertip blood flow in Raynaud’s phenomenon. New England Journal of Medicine, 1971, 285, 259–263.CrossRefGoogle Scholar
  8. Cohen, R., & Coffman, J. Beta-adrenergic vasodilator mechanism in the finger. Clinical Research, 1980, 28, 16lA.Google Scholar
  9. Dabich, L., Bookstein, J., Sweifler, A., & Zarafonetis, J. Digital arteries in patients with scleroderma. Archives of Internal Medicine, 1972, 130, 708–714.CrossRefGoogle Scholar
  10. Emmannel, G., Saroja, D., Gopinathan, K., & Kaplan, D. Environmental factors and the diffusing capacity of the lungs in progressive systemic sclerosis. Chest, 1976 (suppl.) 2, 304–307.Google Scholar
  11. Freedman, R., & Ianni, P. Voluntary control of skin temperature. Psychophysiology, 1981, 18, 197.Google Scholar
  12. Freedman, R., Ianni. P., Hale, P., & Lynn, S. Treatment of Raynaud’s phenomenon with biofeedback and cold sensitization. Psychophysiology, 1979, 16, 182.Google Scholar
  13. Freedman, R., Lynn, S., & lanni, P. Raynaud’s disease. In F. Keefe & J. Blumenthal (eds.) Assessment Strategies in Behavioral Medicine. New York: Grune and Stratton, in press.Google Scholar
  14. Freedman, R., Lynn, S., lanni. P., & Hale, P. Biofeedback treatment of Raynaud’s disease and phenomenon. Biofeedback and Self-Regulation, 1981, 6, 355–365.CrossRefGoogle Scholar
  15. Fries, J. Physiologic studies in systemic sclerosis (scleroderma). Archives of Internal Medicine, 1969, 123, 22–25.CrossRefGoogle Scholar
  16. Fries, J. The microvascular pathogenesis of scleroderma: an hypothesis. Annals of Internal Medicine, 1979, 9, 788–789.ADSGoogle Scholar
  17. Fries, J., Hoopes, J., & Shulman, L. Reciprocal skin grafts in systemic sclerosis (scleroderma). Arthritis and Rheumatology, 1971 14, 571–578.CrossRefGoogle Scholar
  18. Gladman, D., Gordon, D., Urowitz, M., & Levy, H. Pericardial fluid analysis in scleroderma. American Journal of Medicine, 1976, 60, 1064–1068.CrossRefGoogle Scholar
  19. Guttadauria, M., Ellman, H., & Kaplan, D. Progressive systemic sclerosis: pulmonary involvement. Clinics in Rhematic Diseases, 1979, 5 (1), 151–166.Google Scholar
  20. Keefe, F.J. Biofeedback vs. instructional control in skin temperature. Journal of Behavior Medicine, 1978, 1, 383–390.CrossRefGoogle Scholar
  21. Kristensen, J. Local regulation of blood flow in cutaneous and subcutaneous tissue in patients with generalized scleroderma. Acta Dermato-Venerologica, 1980 (suppl.) 90, 1–39.Google Scholar
  22. Kurland, L., Hauser, W., Ferguson, R., & Holley, K. Epidemiologic features of connective tissue diseases in Rochester, Minnesota. Mayo Clinic Proceedings, 1969, 44, 649–663.Google Scholar
  23. LeRoy, E. Scleroderma (systemic sclerosis). In W. Kelley, E. Harris, S. Ruddy, & C. Sledge,(eds.) Textbook of Rheumatology. Philadelphia: Saunders, 1981.Google Scholar
  24. LeRoy, E., Downey, J., & Cannon, P. Skin capillary blood flow in scleroderma. Journal of Clinical Investigation, 1971, 50, 930–939.CrossRefGoogle Scholar
  25. Lewis, T. Experiments relating to the peripheral mechanism involved in spasmodic arrest of the circulation in the fingers, a variety of Raynaud’s disease. Heart, 1929, 15, 7–101.Google Scholar
  26. McGrath, M., Peek, R., & Penny, R. Blood hyperviscosity with reduced skin blood flow in scleroderma. Annals of the Rheumatic Diseases, 1977, 36, 569–574.CrossRefGoogle Scholar
  27. Maricq, H., Downey, J., & LeRoy, E. Standstill of nailfold capillary blood flow during cooling in scleroderma and Raynaud’s syndrome. Blood Vessels, 1976, 13, 338–349.Google Scholar
  28. Maricq, H., & LeRoy, E. Patterns of finger capillary abnormalities in connective tissue disease by “widefield” microscopy. Arthritis and Rheumatism, 1973, 16, 619–628.CrossRefGoogle Scholar
  29. Maricq, H., and LeRoy, E. Progressive systemic sclerosis: disorders of the microcirculation. Clinics in Rheumatic Diseases, 1979, 5 (1), 81–102.Google Scholar
  30. Maricq, H., LeRoy, E., D’Angelo, W., Medsger, T., Rodnan, G., Sharp, G., & Wolfe, J. Diagnostic potential of in vivo capillary microscopy in scleroderma and related disorders. Arthritis and Rheumatism, 1980, 61, 862–870.Google Scholar
  31. May, D., & Weber, C. Temperature feedback training for symptom reduction in primary and secondary Raynaud’s disease. Biofeedback and Self-Regulation, 1976, 1, 317.Google Scholar
  32. Medsger, T., & Masi, A. Epidemiology of systemic sclerosis (scleroderma). Annals of Internal Medicine, 1971, 74, 714–721.Google Scholar
  33. Medsger, T., & Masi, A. Epidemilogy of progressive systemic sclerosis. Clinics in Rheumatic Diseases, 1979, 5 (1): 15–25.Google Scholar
  34. Medsger, T., Masi, A., Rodnan, G., Benedek, T., & Robinson, H. Survival with systemic sclerosis (scleroderma) Annals of Internal Medicine, 1971, 75, 369–376.Google Scholar
  35. Myers, A. Progressive systemic sclerosis: gastrointestinal involvement. Clinics in Rheumatic Diseases, 1979, 5 (1), 115–129.Google Scholar
  36. Nilsen, K. Assessment of cold sensitivity in Raynaud’s phenomenon associated with scleroderma. Microvascular Research, 1978, 15, 251–256.CrossRefGoogle Scholar
  37. Peacock, J.H. Peripheral venous blood concentrations of epinephrine and norepinephrine in primary Raynaud’s disease. Circulation Research, 1959, 1, 821–827.Google Scholar
  38. Rodnan, G. Progressive systemic sclerosis (scleroderma). In D. McCarty (ed.) Arthritis and Allied Conditions. Philadelphia: Lea and Febiger, 1979a.Google Scholar
  39. Rodnan, G. Progressive systemic sclerosis: clinical feature and pathogenesis of cutaneous involvement (scleroderma). Clinics in Rheumatic Diseases, 1979b, 5 (1), 49–79.Google Scholar
  40. Rodnan, G., Lipinski, E., & Luksick, J. Skin thickness and collagen content in progressive systemic scleorsis and localized scleroderma. Arthritis and Rheumatism, 1979, 22, 130–140.CrossRefGoogle Scholar
  41. Rosch, J., Porter, J., & Gralino, B. Cyrodynamic hand angiography in the diagnosis and management of Raynaud’s syndrome. Circulation, 1977, 55, 807–814.Google Scholar
  42. Sapira, J., Rodnan, G., Scheib, E., Klaniecki, T., & Rizk, M. Studies of endogeneous catecholamines secondary to progressive systemic sclerosis. American Journal of Medicine, 1972, 52, 330–337.CrossRefGoogle Scholar
  43. Surwit, R., Allen, L., Kuhn, C., Gilgor, R., Duvic, M., Schanberg, J., & Williams, R. Neuroendocrine correlates of Raynaud’s disease and Raynaud’s phenomenon, Psychophysiology, 1981, 18, 204.Google Scholar
  44. Surwit, R., & Fenton, C. Feedback and instructions in the control of digital skin temperature. Psychophysiology, 1980, 17, 129–132.CrossRefGoogle Scholar
  45. Tan, E., Rodnan, G., Garcia, 1., Morin, Y., Fritzler, M., and Peebles, C., Diversity of antinuclear antibodies in progressive systemic sclerosis. Arthritis & Rheumatism, 1980, 23, 617–625.CrossRefGoogle Scholar
  46. Turner, R., Lipshutz, W., Miller, W., Rittenberg, G., Schumacher, H., & Cohen, S. Esophageal dysfunction in collagen disease. American Journal of Medical Sciences, 1973, 265, 191–199.CrossRefGoogle Scholar
  47. Wouda, A. Raynaud’s phenomenon. Acta Medica Scandanavica, 1977, 201, 519–523.CrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1982

Authors and Affiliations

  • Robert Freedman
    • 1
  • Paul Wenig
    • 1
  1. 1.Lafayette Clinic and Wayne State UniversityDetroitUSA

Personalised recommendations