Skip to main content
SpringerLink
Log in

Systemic Vasospasm

  • Chapter
  • First Online:
Raynaud’s Phenomenon

  • 1615 Accesses

Abstract

Raynaud phenomenon (RP) is thought to be an exaggerated vasoactive response to environmental temperatures and emotional stress. Abnormal vascular reactivity is also the presumed mechanism underlying several common and uncommon disorders such as migraine headaches, preeclampsia, and variant angina. While epidemiologic studies (case reports, case series, and some controlled studies) have linked RP and these conditions, there is yet to be any clearly defined systemic vasoreactivity syndrome. Raynaud phenomenon is also a hallmark feature of systemic sclerosis, a disease with components of both vasospasm and structural small vessel disease that may lead to tissue ischemia of the digits. Whether other organ systems in patients with scleroderma also have a component of vasospasm as either an inciting event or contributing factor to damage is yet to be clearly defined. This chapter first examines the possible role of vasospasm in scleroderma outside of the peripheral circulation and RP in playing a role in the pathogenesis of scleroderma renal crisis, pulmonary hypertension, and cardiac disease. This chapter then examines the evidence that may exist linking RP with systemic vascular disorders.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

SSc:

Scleroderma

RP:

Raynaud phenomenon

ECG:

Electrocardiogram

DLCO:

Carbon monoxide

CGRP:

Calcitonin gene-related protein

MINC:

Myocardial infarction with normal coronary arteries

References

  1. Al-Dhaher FF, Pope JE, Ouimet JM. Determinants of morbidity and mortality of systemic sclerosis in Canada. Semin Arthritis Rheum. 2010;39(4):269–77.

    Article  PubMed  Google Scholar 

  2. Allanore Y, Meune C, Vonk MC, Airo P, Hachulla E, Caramaschi P, et al. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patients. Ann Rheum Dis. 2009;10. Epub 2009/03/13. Eng.

    Google Scholar 

  3. Steen VD, Follansbee WP, Conte CG, Medsger Jr TA. Thallium perfusion defects predict subsequent cardiac dysfunction in patients with systemic sclerosis. Arthritis Rheum. 1996;39(4):677–81. PubMed PMID: 8630120.

    Article  PubMed  CAS  Google Scholar 

  4. Khurma V, Meyer C, Park GS, McMahon M, Lin J, Singh RR, et al. A pilot study of subclinical coronary atherosclerosis in systemic sclerosis: coronary artery calcification in cases and controls. Arthritis Rheum. 2008;59(4):591–7. PubMed PMID: 18383403.

    Article  PubMed  CAS  Google Scholar 

  5. Vesely MR, Dilsizian V. Microvascular angina: assessment of coronary blood flow, flow reserve, and metabolism. Curr Cardiol Rep. 2011;13(2):151–8. PubMed PMID: 21234817.

    Article  PubMed  Google Scholar 

  6. Siegel RJ, O’Connor B, Mena I, Criley JM. Left ventricular function at rest and during Raynaud’s phenomenon in patients with scleroderma. Am Heart J. 1984;108(6):1469–76. PubMed PMID: 6507243.

    Article  PubMed  CAS  Google Scholar 

  7. Alexander EL, Firestein GS, Weiss JL, Heuser RR, Leitl G, Wagner Jr HN, et al. Reversible cold-induced abnormalities in myocardial perfusion and function in systemic sclerosis. Ann Intern Med. 1986;105(5):661–8. PubMed PMID: 3767147.

    Article  PubMed  CAS  Google Scholar 

  8. Candell-Riera J, Armadans-Gil L, Simeon CP, Castell-Conesa J, Fonollosa-Pla V, Garcia-del-Castillo H, et al. Comprehensive noninvasive assessment of cardiac involvement in limited systemic sclerosis. Arthritis Rheum. 1996;39(7):1138–45. PubMed PMID: 8670322.

    Article  PubMed  CAS  Google Scholar 

  9. Lekakis J, Mavrikakis M, Emmanuel M, Prassopoulos V, Papazoglou S, Papamichael C, et al. Cold-induced coronary Raynaud’s phenomenon in patients with systemic sclerosis. Clin Exp Rheumatol. 1998;16(2):135–40. PubMed PMID: 9536388.

    PubMed  CAS  Google Scholar 

  10. el Tarek G, Yasser AE, Gheita T. Coronary angiographic findings in asymptomatic systemic sclerosis. Clin Rheumatol. 2006;25(4):487–90. PubMed PMID: 16440131.

    Article  Google Scholar 

  11. Parks JL, Taylor MH, Parks LP, Silver RM. Systemic sclerosis and the heart. Rheum Dis Clin North Am. 2014;40(1):87–102. PubMed PMID: 24268011.

    Article  PubMed  Google Scholar 

  12. Mizuno R, Fujimoto S, Saito Y, Nakamura S. Cardiac Raynaud’s phenomenon induced by cold provocation as a predictor of long-term left ventricular dysfunction and remodelling in systemic sclerosis: 7-year follow-up study. Eur J Heart Fail. 2010;12(3):268–75. PubMed PMID: 20071354.

    Article  PubMed  Google Scholar 

  13. Kahan A, Devaux JY, Amor B, Menkes CJ, Weber S, Nitenberg A, et al. Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis. N Engl J Med. 1986;314(22):1397–402.

    Article  PubMed  CAS  Google Scholar 

  14. Sokoloff L. Some aspects of the pathology of collagen diseases. Bull N Y Acad Med. 1956;32(10):760–7. PubMed PMID: 13364587. Pubmed Central PMCID: 1806016.

    PubMed  CAS  PubMed Central  Google Scholar 

  15. Cannon PJ, Hassar M, Case DB, Casarella WJ, Sommers SC, LeRoy EC. The relationship of hypertension and renal failure in scleroderma (progressive systemic sclerosis) to structural and functional abnormalities of the renal cortical circulation. Medicine (Baltimore). 1974;53(1):1–46. PubMed PMID: 4808710.

    Article  CAS  Google Scholar 

  16. Scorza R, Rivolta R, Mascagni B, Berruti V, Bazzi S, Castagnone D, et al. Effect of iloprost infusion on the resistance index of renal vessels of patients with systemic sclerosis. J Rheumatol. 1997;24(10):1944–8. PubMed PMID: 9330936.

    PubMed  CAS  Google Scholar 

  17. Rosato E, Cianci R, Barbano B, Menghi G, Gigante A, Rossi C, et al. N-acetylcysteine infusion reduces the resistance index of renal artery in the early stage of systemic sclerosis. Acta Pharmacol Sin. 2009;30(9):1283–8. PubMed PMID: 19730428.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  18. Aikimbaev KS, Canataroglu A, Ozbek S, Usal A. Renal vascular resistance in progressive systemic sclerosis: evaluation with duplex Doppler ultrasound. Angiology. 2001;52(10):697–701. PubMed PMID: 11666134.

    Article  PubMed  CAS  Google Scholar 

  19. Yamauchi K, Arimori S. Alterations of glomerular filtration rate during cold exposure in progressive systemic sclerosis: measurement with technetium-99m DTPA. Jpn J Med. 1990;29(2):208–11. PubMed PMID: 2232369.

    Article  PubMed  CAS  Google Scholar 

  20. Kovalchik MT, Guggenheim SJ, Silverman MH, Robertson JS, Steigerwald JC. The kidney in progressive systemic sclerosis: a prospective study. Ann Intern Med. 1978;89(6):881–7. PubMed PMID: 717985.

    Article  PubMed  CAS  Google Scholar 

  21. Bruyn GA, Van Leeuwen MS. Failure of cold pressor testing to induce changes in plasma renin activity or renal duplex sonography in patients with systemic sclerosis. Ann Rheum Dis. 1992;51(8):1022. PubMed PMID: 1417112.

    PubMed  CAS  Google Scholar 

  22. Rozkovec A, Bernstein R, Asherson RA, Oakley CM. Vascular reactivity and pulmonary hypertension in systemic sclerosis. Arthritis Rheum. 1983;26(8):1037–40. PubMed PMID: 6882479.

    Article  PubMed  CAS  Google Scholar 

  23. Ohar JM, Robichaud AM, Fowler AA, Glauser FL. Increased pulmonary artery pressure in association with Raynaud’s phenomenon. Am J Med. 1986;81(2):361–2. PubMed PMID: 3740093.

    Article  PubMed  CAS  Google Scholar 

  24. Naslund MJ, Pearson TA, Ritter JM. A documented episode of pulmonary vasoconstriction in systemic sclerosis. Johns Hopkins Med J. 1981;148(2):78–80. PubMed PMID: 7206403.

    PubMed  CAS  Google Scholar 

  25. Morgan JM, Griffiths M, du Bois RM, Evans TW. Hypoxic pulmonary vasoconstriction in systemic sclerosis and primary pulmonary hypertension. Chest. 1991;99(3):551–6. PubMed PMID: 1899817.

    Article  PubMed  CAS  Google Scholar 

  26. Yamauchi K, Arimori S. Alteration in pulmonary blood flow induced by cold exposure to the hand in progressive systemic sclerosis. Jpn J Med. 1989;28(4):506–9. PubMed PMID: 2810923.

    Article  PubMed  CAS  Google Scholar 

  27. Wise RA, Wigley F, Newball HH, Stevens MB. The effect of cold exposure on diffusing capacity in patients with Raynaud’s phenomenon. Chest. 1982;81(6):695–8. PubMed PMID: 7075304.

    Article  PubMed  CAS  Google Scholar 

  28. Miller MJ. Effect of the cold pressor test on diffusing capacity. Comparison of normal subjects and those with Raynaud's disease and progressive systemic sclerosis. Chest. 1983;84(3):264–6. PubMed PMID: 6884100.

    Article  PubMed  CAS  Google Scholar 

  29. Gastaud M, Dolisi C, Bermon S, Gaudin P, Defauw G, Ardisson JL. Short-term effect of cold provocation on single-breath carbon monoxide diffusing capacity in subjects with and without Raynaud’s phenomenon. Clin Exp Rheumatol. 1995;13(5):617–21. PubMed PMID: 8575140.

    PubMed  CAS  Google Scholar 

  30. Fahey PJ, Utell MJ, Condemi JJ, Green R, Hyde RW. Raynaud’s phenomenon of the lung. Am J Med. 1984;76(2):263–9. PubMed PMID: 6695950.

    Article  PubMed  CAS  Google Scholar 

  31. Sfikakis PP, Kyriakidis M, Vergos C, Papazoglou S, Georgiakodis F, Toutouzas P, et al. Diffusing capacity of the lung and nifedipine in systemic sclerosis. Arthritis Rheum. 1990;33(11):1634–9. PubMed PMID: 2242061.

    Article  PubMed  CAS  Google Scholar 

  32. Shuck JW, Oetgen WJ, Tesar JT. Pulmonary vascular response during Raynaud’s phenomenon in progressive systemic sclerosis. Am J Med. 1985;78(2):221–7. PubMed PMID: 3970048.

    Article  PubMed  CAS  Google Scholar 

  33. Mukerjee D, Yap LB, Ong V, Denton CP, Howells K, Black CM, et al. The myth of pulmonary Raynaud’s phenomenon: the contribution of pulmonary arterial vasospasm in patients with systemic sclerosis related pulmonary arterial hypertension. Ann Rheum Dis. 2004;63(12):1627–31. PubMed PMID: 15547087.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  34. Nishida A, Kaiya H, Uematsu M, Maeda M, Mori S, Wakabayashi S. Transient global amnesia and Raynaud’s phenomenon in scleroderma. Acta Neurol Scand. 1990;81(6):550–2. PubMed PMID: 2220314.

    Article  PubMed  CAS  Google Scholar 

  35. Grecco MP, Pieroni M, Otero M, Ferreiro JL, Figuerola ML. Sporadic hemiplegic migraine and CREST syndrome. J Headache Pain. 2010;11(2):171–3. PubMed PMID: 20130953. Pubmed Central PMCID: 3452292.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Ferraccioli G, Di Poi E, Di Gregorio F, Giacomuzzi F, Guerra U. Changes in regional cerebral blood flow after a cold hand test in systemic lupus erythematosus patients with Raynaud’s syndrome. Lancet. 1999;354(9196):2135–6. PubMed PMID: 10609825.

    Article  PubMed  CAS  Google Scholar 

  37. Terrier B, Charbonneau F, Touze E, Berezne A, Pagnoux C, Silvera S, et al. Cerebral vasculopathy is associated with severe vascular manifestations in systemic sclerosis. J Rheumatol. 2009;36(7):1486–94. PubMed PMID: 19531752.

    Article  PubMed  Google Scholar 

  38. Hong P, Pope JE, Ouimet JM, Rullan E, Seibold JR. Erectile dysfunction associated with scleroderma: a case-control study of men with scleroderma and rheumatoid arthritis. J Rheumatol. 2004;31(3):508–13. PubMed PMID: 14994396.

    PubMed  Google Scholar 

  39. Merla A, Romani GL, Tangherlini A, Di Romualdo S, Proietti M, Rosato E, et al. Penile cutaneous temperature in systemic sclerosis: a thermal imaging study. Int J Immunopathol Pharmacol. 2007;20(1):139–44. PubMed PMID: 17346437.

    PubMed  CAS  Google Scholar 

  40. van Wyk L, van der Marel J, Schuerwegh AJ, Schouffoer AA, Voskuyl AE, Huizinga TW, et al. Increased incidence of pregnancy complications in women who later develop scleroderma: a case control study. Arthritis Res Ther. 2011;13(6):R183. PubMed PMID: 22053948. Pubmed Central PMCID: 3334631.

    Article  PubMed  PubMed Central  Google Scholar 

  41. Hartgill TW, Pirhonen J. Blood pressure rises more in pre-eclampsia than normal pregnancy when acral skin is locally cooled. Hypertens Pregnancy. 2013;32(4):340–54. PubMed PMID: 23844619.

    Article  PubMed  CAS  Google Scholar 

  42. de Trafford JC, Lafferty K, Potter CE, Roberts VC, Cotton LT. An epidemiological survey of Raynaud’s phenomenon. Eur J Vasc Surg. 1988;2(3):167–70. PubMed PMID: 3410065.

    Article  PubMed  Google Scholar 

  43. Zahavi I, Chagnac A, Hering R, Davidovich S, Kuritzky A. Prevalence of Raynaud’s phenomenon in patients with migraine. Arch Intern Med. 1984;144(4):742–4.

    Article  PubMed  CAS  Google Scholar 

  44. Leppert J, Aberg H, Ringqvist I, Sorensson S. Raynaud’s phenomenon in a female population: prevalence and association with other conditions. Angiology. 1987;38(12):871–7. PubMed PMID: 3425973.

    Article  PubMed  CAS  Google Scholar 

  45. O'Keeffe ST, Tsapatsaris NP, Beetham Jr WP. Association between Raynaud’s phenomenon and migraine in a random population of hospital employees. J Rheumatol. 1993;20(7):1187–8. PubMed PMID: 8371215.

    PubMed  Google Scholar 

  46. Pal B, Gibson C, Passmore J, Griffiths ID, Dick WC. A study of headaches and migraine in Sjogren’s syndrome and other rheumatic disorders. Ann Rheum Dis. 1989;48(4):312–6. PubMed PMID: 2712611. Pubmed Central PMCID: 1003747.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  47. Amin FM, Asghar MS, Hougaard A, Hansen AE, Larsen VA, de Koning PJ, et al. Magnetic resonance angiography of intracranial and extracranial arteries in patients with spontaneous migraine without aura: a cross-sectional study. Lancet Neurol. 2013;12(5):454–61. PubMed PMID: 23578775.

    Article  PubMed  Google Scholar 

  48. Tunis MM, Wolff HG. Analysis of cranial artery pulse waves in patients with vascular headache of the migraine type. Am J Med Sci. 1952;224(5):565–8. PubMed PMID: 12985578.

    Article  PubMed  CAS  Google Scholar 

  49. Mirza AF, Mo J, Holt JL, Kairalla JA, Heft MW, Ding M, et al. Is there a relationship between throbbing pain and arterial pulsations? J Neurosci. 2012;32(22):7572–6. PubMed PMID: 22649235. Pubmed Central PMCID: 3376713.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  50. Hoffmann J, Goadsby PJ. Emerging targets in migraine. CNS Drugs. 2014;28(1):11–7. PubMed PMID: 24318669.

    Article  PubMed  CAS  Google Scholar 

  51. Bunker CB, Terenghi G, Springall DR, Polak JM, Dowd PM. Deficiency of calcitonin gene-related peptide in Raynaud’s phenomenon. Lancet. 1990;336(8730):1530–3. PubMed PMID: 1979366.

    Article  PubMed  CAS  Google Scholar 

  52. Ammann P, Marschall S, Kraus M, Schmid L, Angehrn W, Krapf R, et al. Characteristics and prognosis of myocardial infarction in patients with normal coronary arteries. Chest. 2000;117(2):333–8. PubMed PMID: 10669671.

    Article  PubMed  CAS  Google Scholar 

  53. Koh KK, Kim SH, Lee KH, Kwon KS, Kim EJ, Baik SH, et al. Does prevalence of migraine and Raynaud’s phenomenon also increase in Korean patients with proven variant angina? Int J Cardiol. 1995;51(1):37–46. PubMed PMID: 8522395.

    Article  PubMed  CAS  Google Scholar 

  54. O’Keeffe ST, Tsapatsaris NP, Beetham Jr WP. Increased prevalence of migraine and chest pain in patients with primary Raynaud disease. Ann Intern Med. 1992;116(12 Pt 1):985–9. PubMed PMID: 1586109.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Suite 4000, Mason F. Lord Building, Center Tower, Baltimore, MD, 21224, USA

    Laura K. Hummers M.D., Sc.M.

Authors
  1. Laura K. Hummers M.D., Sc.M.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Laura K. Hummers M.D., Sc.M. .

Editor information

Editors and Affiliations

  1. School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  2. Department of Rheumatology, The University of Manchester, Manchester, United Kingdom

    Ariane L. Herrick

  3. Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medic, Baltimore, Maryland, USA

    Nicholas A. Flavahan

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media New York

About this chapter

Cite this chapter

Hummers, L.K. (2015). Systemic Vasospasm. In: Wigley, F., Herrick, A., Flavahan, N. (eds) Raynaud’s Phenomenon. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1526-2_16

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-1526-2_16

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-1525-5

  • Online ISBN: 978-1-4939-1526-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation