Skip to main content

Advertisement

Log in

Non-invasive diagnostic and functional evaluation of cardiac involvement in patients with systemic sclerosis

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

Abstract

Elevated serum brain natriuretic peptide (BNP) released from myocytes of ventricles upon stretch have been found in patients with isolated right ventricular (RV) pressure overload. However, limited data suggest that serum BNP may be elevated in systemic sclerosis (SSc) patients, especially with RV dysfunction. We assessed serum N-terminal proBNP (NT-proBNP) in SSc and evaluated whether it reflects the severity of RV overload. We prospectively studied 51 consecutive patients (47F, mean age 53.3 ± 15.2 years) with SSc (mean disease duration 9 ± 12.4 years). The control group formed 31 healthy subjects (27F, mean age 52.6 ± 12.1 years). NT-proBNP level, 6-minute walking test (6MWT), and transthoracic echocardiography (TTE) for the assessment of RV overload were performed. Serum NT-proBNP exceeded the reference value of 125 pg/mL in 31 (61%) SSc patients. The mean serum log NT-proBNP concentration in SSc was higher than in controls (2.138 ± 0.527 vs. 1.634 ± 0.420 pg/mL, p < 0.001). 13 (25%) SSc patients have tricuspid regurgitation peak gradient (TRPG) exceeding 31 mmHg reflecting pulmonary arterial hypertension (PAH). The SSc presented other echocardiographic signs of RV overload. Mean 6MWT distance was shorter in SSc than in controls (528 ± 100 vs. 617 ± 80 m, p < 0.001). NT-proBNP level correlated positively with TRPG, RV diameter, RV Tei index and negatively with 6MWT distance. ROC analysis identified >115 pg/ml as the best NT-proBNP threshold predicting PAH for SSc patients (sensitivity 92%, specificity 44%). Results of our study suggest that NT-proBNP measurement is a useful screening method for PAH in SSc patients. Since elevated plasma NT-proBNP level reflects the degree of right ventricular overload and limitation of exercise capacity, abnormal NT-proBNP levels should imply further evaluation including echocardiography.

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

Similar content being viewed by others

Abbreviations

AcT:

right ventricular acceleration time

BNP:

brain natriuretic peptide

DLCO:

diffusion capacity of the lung for carbon monoxide

EF:

left ventricular ejection fraction

FEV1 :

forced expiratory volume in 1 s

FVC:

forced vital capacity

HR:

heart rate

IVC:

diameter of inferior vena cava

LV:

left ventricle

NT-proBNP:

N-terminal proBNP

PAH:

pulmonary arterial hypertension

RV:

right ventricle

SSc:

systemic sclerosis

TLC:

total lung capacity

TRPG:

tricuspid regurgitation peak gradient

TTE:

transthoracic echocardiography

6MWT:

6-minute walking test

Δ sat:

resting oxygen saturation − oxygen saturation after 6MWT

References

  1. Wigley FM (1998) Clinical features of systemic sclerosis. In: Klippel JH, Dieppe PA (eds) Rheumatology. Mosby, London, NY, pp 1–14

    Google Scholar 

  2. LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma: classification, subset and pathogenesis. J Rheumatol 15:202–205

    PubMed  CAS  Google Scholar 

  3. Koh E, Lee P, Gladman DD (1996) Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 35:989–993

    Article  PubMed  CAS  Google Scholar 

  4. Schachna L, Wigley FM, Chang B et al (2003) Age and risk of pulmonary arterial hypertension in scleroderma. Chest 124:2098–2104

    Article  PubMed  Google Scholar 

  5. Morelli S, De Marzio P, Valesini G et al (1993) Pulmonary hypertension in systemic sclerosis. G Ital Cardiol 23:871–876

    PubMed  CAS  Google Scholar 

  6. Hachulla E, Gressin V, Guillevin L et al (2005) Early detection of pulmonary arterial hypertension in systemic sclerosis. Arthritis Rheum 52:3792–3800

    Article  PubMed  Google Scholar 

  7. Galie N, Manes A, Farahani KV et al (2005) Pulmonary arterial hypertension associated to connective tissue diseases. Lupus 14:713–717

    Article  PubMed  CAS  Google Scholar 

  8. Nagaya N, Ando M, Oya H et al (2002) Plasma brain natriuretic peptide as a noninvasive marker for efficacy of pulmonary thromboendarterectomy. Ann Thorac Surg 74:180–184

    Article  PubMed  Google Scholar 

  9. Nagaya N, Nishikimi T, Uematsu M et al (2000) Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 102:865–870

    PubMed  CAS  Google Scholar 

  10. Mukerjee D, Yap L, Holmes AM et al (2003) Significance of plasma N-terminal pro-brain natriuretic peptide in patients with systemic sclerosis-related pulmonary arterial hypertension. Respir Med 97:1230–1236

    Article  PubMed  CAS  Google Scholar 

  11. Emdin M, Marini C, Passino C (2004) Right ventricular overload and cardiovascular neuroendocrine derangement in systemic sclerosis. Eur Heart J 6(suppl F):68–73

    Google Scholar 

  12. Williams MH, Handler CE, Akram R et al (2006) Role of N-terminal brain natriuretic peptide (NT-proBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 27:1485–1494

    Article  PubMed  CAS  Google Scholar 

  13. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581–590

    Article  Google Scholar 

  14. Torbicki A, Kurzyna M, Ciurzyński M et al (1999) Proximal pulmonary emboli modify right ventricular ejection pattern. Eur Respir J 13:616–621

    Article  PubMed  CAS  Google Scholar 

  15. Galie N, Torbicki A, Barst R et al (2004) Guidelines on diagnosis and treatment of pulmonary arterial hypertension. Eur Heart J 25:2243–2278

    Article  PubMed  Google Scholar 

  16. Tei C, Dujardin KS, Hodge DO et al (1996) Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr 9:838–847

    Article  PubMed  CAS  Google Scholar 

  17. Denton CP, Cailes JB, Phillips, GD et al (1997) Comparison of Doppler echocardiography and right heart catheterization in systemic sclerosis. Br J Rheumatol 36:239–243

    Article  PubMed  CAS  Google Scholar 

  18. Mukerjee D, St George D, Knight C et al (2004) Echocardiography and pulmonary function as screening tests for pulmonary arterial hypertension in systemic sclerosis. Rheumatology 43:461–466

    Article  PubMed  CAS  Google Scholar 

  19. McGoon M, Gutterman D, Steen V et al (2004) Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126:145–345

    Article  Google Scholar 

  20. Pruszczyk P, Kostrubiec M, Bochowicz A et al (2003) N-terminal pro-brain natriuretic peptide in patients with acute pulmonary embolism. Eur Resp J 22:649–653

    Article  CAS  Google Scholar 

  21. Murata I, Takenaka K, Shinohara S (1998) Diversity of myocardial involvement in systemic sclerosis: an 8-year study of 95 Japanese patients. Am Heart J 135:960–969

    Article  PubMed  CAS  Google Scholar 

  22. Jacobsen S, Ullman S, Shen GQ et al (2001) Influence of clinical features, serum antinuclear antibodies, and lung function on survival of patients with systemic sclerosis. J Rheumatol 28:2454–2459

    PubMed  CAS  Google Scholar 

  23. Steen V, Medsger TA Jr (2003) Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 48:516–522

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michał Ciurzyński.

Additional information

This study was partially supported by grant KBN N402 085034.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ciurzyński, M., Bienias, P., Lichodziejewska, B. et al. Non-invasive diagnostic and functional evaluation of cardiac involvement in patients with systemic sclerosis. Clin Rheumatol 27, 991–997 (2008). https://doi.org/10.1007/s10067-008-0837-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-008-0837-9

Keywords

Navigation