Skip to main content
SpringerLink
Log in

Autonomic dysfunction and microvascular damage in systemic sclerosis

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular damage and interstizial fibrosis of many organs. Our interest was focused on the evaluation of cardiac autonomic function by measurements of heart rate variability (HRV) and microvascular damage detected by nailfold capillaroscopy (NC) in SSc patients. We examined 25 consecutive outpatients affected by systemic sclerosis and 25 healthy controls. Exclusion criteria were the presence of cardiac disease, hypertension, diabetes mellitus, or neurological diseases. All subjects underwent 24-h ambulatory ECG Holter recording and NC examination. Heart rate variability was evaluated in the time domain, using appropriate software, computing the time series of all normal-to-normal (NN) QRS intervals throughout the 24-h recording period. A semiquantitative rating scale was adopted to score the NC abnormalities, as well as a rating system for avascular areas and morphological NC patterns. In SSc patients, HRV analysis showed significantly lower values of SDNN (standard deviation of all NN intervals) (p = 0.009), SDANN (standard deviation of the averages of NN intervals in all 5-min segments of the entire recording) (p = 0.01), and pNN50 (the percentage of adjacent NN intervals that differed by more than 50 ms) (p = 0.02), compared to the control group. These parameters in SSc patients significantly decreased with the worsening of semiquantitative capillaroscopy score. In conclusion, an abnormal autonomic nervous control of the heart might contribute to identify subclinical cardiac involvement in SSc patients. The coexistence of autonomic dysfunction with a more severe microvascular damage could be considered a potential prognostic tool in the identification of those patients particularly at risk for cardiac mortality.

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

Fig. 1

Similar content being viewed by others

References

  1. LeRoy EC (1996) Systemic sclerosis. A vascular perspective. Rheum Dis Clin North Am 22:675–694

    Article  PubMed  CAS  Google Scholar 

  2. Kahaleh BM (1994) Raynaud’s phenomenon and vascular disease in scleroderma. Curr Opin Rheumatol 6:621–627

    Article  PubMed  CAS  Google Scholar 

  3. Dessein PH, Joffe BI, Metz RM, Millar DL, Lawson M, Stanwix AE (1992) Autonomic dysfunction in systemic sclerosis: sympathetic overactivity and instability. Am J Med 93:143–150

    Article  PubMed  CAS  Google Scholar 

  4. Ferri C, Emdin M, Giuggioli D, Carpeggiani C, Maielli M, Varga A, Michelassi C, Pasero G, L’Abbate A (1997) Autonomic dysfunction in systemic sclerosis: time and frequency domain 24 hour heart rate variability analysis. Br J Rheumatol 36:669–676

    Article  PubMed  CAS  Google Scholar 

  5. Morelli S, Piccirillo G, Fimognani F, Sgreccia A, Ferrante L, Morabito G, De Marzio P, Valesini G, Marigliano V (1996) Twenty-four hour heart period variability in systemic sclerosis. J Rheumatol 23:643–645

    PubMed  CAS  Google Scholar 

  6. Akselrod S, Gordon D, Ubel FA, Shannon DC, Barger AC, Cohen RJ (1981) Power spectrum analysis of heart rate fluctuation: a quantitative probe of beat-to-beat cardiovascular control. Science 213:220–222

    Article  PubMed  CAS  Google Scholar 

  7. Maricq HR, Harper FE, Khan MM, Leroy EC (1983) Microvascular abnormalities as possible predictor of disease subset in Raynaud’s phenomenon and early connective tissue disease. Clin Exp Rheumatol 1:195–205

    PubMed  CAS  Google Scholar 

  8. Masi AT, Medsger TA, Rodnam GP, Fries JR, Altman RD, Brown BW (1980) Preliminary criteria fort he classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590

    Article  Google Scholar 

  9. Task Force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology (1996) Heart rate variability: standards of measurement, physiological interpretation and clinical use. Circulation 93:1043–1065

    Google Scholar 

  10. Stein PK (2002) Assessing heart variability from real—world Holter reports. Card Electrophysiol Rev 6:239–244

    Article  PubMed  Google Scholar 

  11. Kabasakal Y, Elvins DM, Ring EF, McHugh NJ (1996) Quantitative nailfold capillaroscopy findings in a population with connective tissue disease and in normal healthy controls. Ann Rheum Dis 55:507–512

    Article  PubMed  CAS  Google Scholar 

  12. Riccieri V, Rinaldi T, Spadaro A, Scrivo R, Ceccarelli F, Di Franco M, Taccari E, Valesini G (2003) Interleukin 13 in systemic sclerosis: relationship to nailfold capillaroscopy abnormalities. Clin Rheumatol 22:102–106

    Article  PubMed  CAS  Google Scholar 

  13. Maricq HR (1981) Wide-field capillary microscopy: technique and rating scale for abnormalities seen in scleroderma and related disorders. Arthritis Rheum 24:1159–1165

    Article  PubMed  CAS  Google Scholar 

  14. Cutolo M, Sulli A, Pizzorni C, Accardo S (2000) Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol 27:155–160

    PubMed  CAS  Google Scholar 

  15. Lee P, Leung FYK, Alderice C, Amstrong SK (1983) Nailfold capillary microscopy in the connective tissue diseases: a semiquantitative assessment. J Rheumatol 10:930–938

    PubMed  CAS  Google Scholar 

  16. Kleiger RE, Miller JP, Bigger JT Jr, Moss AJ, Multicenter Post-Infarction Research Group (1987) Decreased heart rate variability and its association with increased mortality after acute myocardial infarction. Am J Cardiol 59:256–262

    Article  PubMed  CAS  Google Scholar 

  17. Saul JP, Arai Y, Berger RD, Lilly LS, Colucci WS, Cohen RJ (1988) Assessment of autonomic regulation in chronic congestive heart failure by heart rate spectral analysis. Am J Cardiol 61:1292–1299

    Article  PubMed  CAS  Google Scholar 

  18. Tsuji H, Venditti FJ Jr, Manders ES, Evans JC, Larson MG, Feldman CL, Levy D (1994) Reduced heart rate variability and mortality risk in an elderly cohort: the Framingham heart study. Circulation 90:878–883

    PubMed  CAS  Google Scholar 

  19. Pancera P, Sansone S, Presciuttini B, Montagna L, Ceru S, Lunardi C, Lechi A (1999) Autonomic nervous system dysfunction in sclerodermic and primary Raynaud’s phenomenon. Clin Sci 96:49–57

    Article  PubMed  CAS  Google Scholar 

  20. Stacher G, Merio R, Budka C, Schneider C, Smolen J, Tappeiner G (2000) Cardiovascular autonomic function, autoantibodies, and esophageal motor activity in patients with systemic sclerosis and mixed connective tissue disease. J Rheumatol 27:692–697

    PubMed  CAS  Google Scholar 

  21. Kahaleh B, Matucci-Cerinic M (1995) Raynaud’s phenomenon and scleroderma. Disregulated neuroendothelial control of vascular tone. Arthritis Rheum 38:1–4

    Article  PubMed  CAS  Google Scholar 

  22. Herrick LA (2005) Pathogenesis of Raynaud’s phenomenon. Rheumatology 44:587–596

    Article  PubMed  CAS  Google Scholar 

  23. Herrick LA (2000) Vascular function in systemic sclerosis. Curr Opin Rheumatol 12:527–533

    Article  PubMed  CAS  Google Scholar 

  24. Ferri C, Giuggioli D, Sebastiani M, Colaci M, Emdim M (2005) Heart involvement and systemic sclerosis. Lupus 14:702–707

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dipartimento di Clinica e Terapia Medica Applicata, UOC di Medicina Interna, Università di Roma “La Sapienza”, Viale del Policlinico no. 155-, 00161, Rome, Italy

    Manuela Di Franco, Valeria Riccieri & Guido Valesini

  2. Unità di Riabilitazione Intensiva Neuromotoria, Ospedale San Giovanni Battista, Ordine di Malta, Rome, Italy

    Michele Paradiso

  3. UOC di Reumatologia, UOC di Medicina Interna, Università di Roma “La Sapienza”, Viale del Policlinico no. 155-, 00161, Rome, Italy

    Stefania Basili & Antonio Mammarella

Authors
  1. Manuela Di Franco
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Michele Paradiso
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Valeria Riccieri
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Stefania Basili
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Antonio Mammarella
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Guido Valesini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Manuela Di Franco.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Di Franco, M., Paradiso, M., Riccieri, V. et al. Autonomic dysfunction and microvascular damage in systemic sclerosis. Clin Rheumatol 26, 1278–1283 (2007). https://doi.org/10.1007/s10067-006-0492-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-006-0492-y

Keywords

Search

Navigation