Skip to main content
Log in

Abnormal cardiac enzymes in systemic sclerosis: a report of four patients and review of the literature

  • Case Based Review
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Cardiac involvement in systemic sclerosis (SSc) is heterogeneous and can include primary involvement of the myocardium, pericardium and coronary arteries or be secondary to cardiac complications of pulmonary and renal disease. Primary cardiac involvement in SSc is uncommon but can result in ventricular dysfunction, organ failure, arrhythmias and death. It can remain clinically silent and the prevalence is likely to be under-reported. We report four cases of SSc associated with a raised serum troponin T (TnT), in a proportion of whom cardiac MRI myocardial abnormalities were detected. These cases highlight the heterogeneity of cardiac involvement in SSc, the role of cardiac MRI and promising biochemical responses to immunosuppression. Cardiac biomarkers such as TnT may be useful screening tools to identify subclinical cardiac disease and assess response to therapeutic intervention.

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

Access this article

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Eisenberg M, Nguyen BY, Karnath B (2008) Clinical features of systemic sclerosis. Hospital Physician 33–38

  2. Weiss SS, Warren E (1943) Scleroderma heart disease, with a consideration of certain other visceral manifestations of scleroderma. Arch Intern Med 71(6):749–776

    Article  Google Scholar 

  3. Champion HC (2008) The heart in scleroderma. Rheum Dis Clin North Am 34(1):181–190

    Article  PubMed Central  PubMed  Google Scholar 

  4. Piper WN, Helwig EB (1955) Progressive systemic sclerosis: visceral manifestations of generalized scleroderma. AMA Arch Derm 72(6):535–546

    Article  CAS  PubMed  Google Scholar 

  5. Medsger TA Jr, Masi AT (1973) Survival with scleroderma. II. A life-table analysis of clinical and demographic factors in 358 male US veteran patients. J ChronicDis 26(10):647–660

    Article  Google Scholar 

  6. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB et al (2005) Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med 118(1):2–10

    Article  PubMed  Google Scholar 

  7. Tyndall AJ, Bannert B, Vonk M et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69(10):1809–1815

    Article  PubMed  Google Scholar 

  8. Katus HA (2008) Development of the cardiac troponin T immunoassay. Clin Chem 54(9):1576–1577

    Article  CAS  PubMed  Google Scholar 

  9. Badsha H, Gunes B, Grossman J et al (1997) Troponin I assessment of cardiac involvement in patients with connective tissue disease and an elevated creatine kinase MB isoform report of four cases and review of the literature. J Clin Rheumatol 3(3):131–134

    Article  CAS  PubMed  Google Scholar 

  10. Montagnana M, Lippi G, Volpe A et al (2006) Evaluation of cardiac laboratory markers in patients with systemic sclerosis. Clin Biochem 39(9):913–917

    Article  CAS  PubMed  Google Scholar 

  11. Gaal J, Hegedus I, Dévényi K et al (1995) Myocardial gallium-67 citrate scintigraphy in patients with systemic sclerosis. Ann Rheum Dis 54(10):856–858

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  12. Smaldone C, Pieroni M, De Santis M et al (2010) Biopsy proven active myocarditis in Systemic Sclerosis patients with recent onset cardiac involvement. J Am Coll Cardiol 55(10)

  13. Clements PJ, Lachenbruch PA, Seibold JR et al (1993) Skin thickness score in systemic sclerosis: an assessment of interobserver variability in 3 independent studies. J Rheumatol 20(11):1892–1896

    CAS  PubMed  Google Scholar 

  14. Dinser R, Frerix M, Meier FM et al (2013) Endocardial and myocardial involvement in systemic sclerosis—is there a relevant inflammatory component? Joint Bone Spine 80(3):320–323

    Article  CAS  PubMed  Google Scholar 

  15. Allanore Y, Meune C (2010) Primary myocardial involvement in systemic sclerosis: evidence for a microvascular origin. Clin Exp Rheumatol 28(5 Suppl 62):S48–S53

    CAS  PubMed  Google Scholar 

  16. Belloli L, Carlo-Stella N, Ciocia G et al (2008) Myocardial involvement in systemic sclerosis. Rheumatology 47(7):1070–1072

    Article  CAS  PubMed  Google Scholar 

  17. Kahan A, Allanore Y (2006) Primary myocardial involvement in systemic sclerosis. Rheumatology 45(suppl 4):iv14-17.

    Google Scholar 

  18. Hachulla AL, Launay D, Gaxotte V et al (2009) Cardiac magnetic resonance imaging in systemic sclerosis: a cross-sectional observational study of 52 patients. Ann Rheum Dis 68(12):1878–1884

    Article  PubMed Central  PubMed  Google Scholar 

  19. Tzelepis GE, Kelekis N, Plastiras SC et al (2007) Pattern and distribution of myocardial fibrosis in systemic sclerosis: a delayed enhanced magnetic resonance imaging study. Arthritis Rheum 56(11):3827–3836

    Article  PubMed  Google Scholar 

Download references

Patient Consent

Written consent for publication was obtained from all patients featured in this report.

Disclosures

None.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to J D Pauling.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Vasta, B., Flower, V., Bucciarelli-Ducci, C. et al. Abnormal cardiac enzymes in systemic sclerosis: a report of four patients and review of the literature. Clin Rheumatol 33, 435–438 (2014). https://doi.org/10.1007/s10067-013-2405-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-013-2405-1

Keywords

Navigation