The Role of EDD (Exercise Capacity, Dyspnea, and Diffusing Capacity of Lungs for Carbon Monoxide) Index in Pulmonary Fibrosis Secondary to Scleroderma

Document Type : Original Article

Authors

1 Rheumatologist, Rheumatology Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

2 Pulmonologist, Lung Disease Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

3 General Practitioner, Lung Disease Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

4 Statisticien, Vice Chancellery of Research, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

5 Radiologist, Lung Disease Research Center, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

Abstract

Introduction: Pulmonary involvement secondary to Systemic Sclerosis (SSc) is the major cause of morbidity and mortality in SSc patients. We designed this study to determine the correlation of important lung function parameters with lung High Resolution CT (HRCT) scan findings.
Materials and Methods: Thirty–two consecutive diffuse SSc patients with pulmonary fibrosis were enrolled in this cross-sectional study. Patients with pulmonary fibrosis secondary to other causes, previous restrictive lung disease, and history of smoking were excluded. Complete lung function evaluation was performed. The EDD (Exercise capacity, Dyspnea, and Diffusing capacity of lungs for carbon monoxide) index was determined. The Warrick score was calculated based on lung HRCT findings.
Results: The mean age of the patients was 39.18 years ±9.39 (SD).Seventeen (53%) patients were in EDD stage 1 (score: 0≤score≤3), 9 patients (28%) in stage 2 (3<score ≤6), and 6 patients (19%) in stage 3(6<score≤9). The mean Warrick score was 10.84±6.94 (SD).There was statistically significant correlation between EDD index and Warrick score (r=0.72, P=0.001).Also there was statistically significant strong correlation between EDD stages and Warrick scores (r=0.8, P=0.002).
Conclusion: The results of this study revealed that EDD may be a valuable representative marker of lung involvement in SSc and in the future, it can be a suitable and safe alternate modality comparing lung HRCT in our clinical practice and close follow-up.

Keywords

References

  1. Khanna D, Clements PJ, Furst DE, Chon Y, Elashoff R, Roth MD, et al. Correlation of the degree of dyspnea with health-related quality of life , functional abilities, and diffusing capacity for carbon monoxide in patients with systemic sclerosis and active alveolitis: results from the Scleroderma Lung Study. Arthritis Rheum. 2005; 52:592-600.
  2. Gourh P, Arnett FC, Assassi S, Tan FK, Huang M, Diekman L, et al. Plasma cytokine profiles in systemic sclerosis: associations with autoantibody subsets and clinical manifestations. Arthritis Res Ther. 2009; 11:R147.
  3. Hassoun PM. Lung involvement in systemic sclerosis. Presse Med. 2011; 40:e3-17.
  4. Silman AJ. Scleroderma. Baillieres Clin Rheumatol. 1995; 9:471-82.
  5. Lonzetti LS, Joyal F, Raynauld JP, Roussin A, Goulet JR, Rich E, et al. Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum. 2001; 44:735-6.
  6. Denton CP, Hachulla E. Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and its implications for screening. Eur Respir Rev. 2011; 20:270-6.
  7. Launay D, Remy-Jardin M, Michon-Pasturel U, Mastora I, Hachulla E, Lambert M, et al. High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis. J Rheumatol. 2006; 33:1789-801.
  8. Alton E, Turner-Warwick M. Lung involvement in scleroderma. In: Jayson MIV, Blac CM, editors. Systemic sclerosis: scleroderma. London: John Wiley; 1988. P. 181-205.
  9. Wells AU, Hansell DM, Ruben MB, King AD, Cramer D, Black CM, et al. Fibrosing alveolitis in systemic sclerosis : indices of lung function in relation to extent of disease on computed tomography. Arthritis Rheum. 1997; 40:1229-36.
  10. Deuschle K, Weinert K, Becker MO, Backhaus M, Huscher D, Riemekasten G. Six-minute walk distance as a marker for disability and complaints in patients with systemic sclerosis. Clin Exp Rheumatol. 2011; 29:S53-9.
  11. Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE, Clements PJ, et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest. 2008; 134:358-67.
  12. Arroliga AC, Podell DN, Matthay RA. Pulmonary manifestations of scleroderma. J Thorac Imaging. 1992; 7:30–45.
  13. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Pölzleitner D, Burghuber OC, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990; 176:755–9.
  14.  Remy-Jardin M, Remy J, Wallaert B, Bataille D, Hatron PY. Pulmonary involvement in progressive systemic sclerosis: sequential evaluation with CT, pulmonary function tests, and bronchoalveolar lavage. Radiology. 1993; 188:499–506.
  15.  Mayberry JP, Primack SL, Müller NL. Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. Radiographics. 2000; 20:1623-35.
  16. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Pölzleitner D, Burghuber OC, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990;176:755-9.
  17. Warrick JH, Bhalla M, Schabel SI, Silver RM. High resolution computed tomography in early scleroderma lung disease. J Rheumatol. 1991; 18:1520-8.
  18. Celli BR, Cote CG, Marin JM, Casanova C, Montes de Oca M, Mendez RA, et al. The body mass index, airflow obstruction, dyspnea and exercise capacity index in chronic obstructive pulmonary disease. N Engl J Med. 2004; 350:1005–12.
  19. Masi AT, Rodnan GP, Medsger Jr TA, Altman R, D’Angelo W, Fries J. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 1980; 23:581-90.
  20. Clements P, Lachenbruch P, Siebold J, White B, Weiner S, Martin R, et al. Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol. 1995; 22:1281-5.
  21. Goundry B, Bell L, Langtree M, Moorthy A. Diagnosis and management of Raynaud’s phenomenon. BMJ. 2012; 344:e289.
  22. Wanger J, Clausen JL, Coates A, Pedersen OF, Brusasco V, Burgos F, et al. Standardisation of the measurement of lung volumes. Eur Respir J. 2005; 26:511-22.
  23. ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories. ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002; 166:111–7.
  24. Assayag D, Kaduri S, Hudson M, Hirsch A, Baron M. High resolution computed tomography scoring  systems for evaluating interstitial lung disease in systemic sclerosis patients. Rheumatol Curr Res. 2012; 2012:S1-3.
  25. Gargani L, Doveri M, D'Errico L, Frassi F, Bazzichi ML, Delle Sedie A, et al. Ultrasound lung comets in systemic sclerosis: a chest sonography hallmark of pulmonary interstitial fibrosis. Rheumatology (Oxford). 2009; 48:1382-7.
  26. Asano Y, Ihn H, Yamane K, Yazawa N, Kubo M, Fujimoto M, et al. Clinical significance of surfactant protein D as a serum marker for evaluating pulmonary fibrosis in patients with systemic sclerosis. Arthritis Rheum. 2001; 44:1363-9.
  27. Garin MC, Highland KB, Silver RM, Strange C. Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol. 2009; 36:330-6.

Journal of Cardio-Thoracic Medicine
Volume 4, Issue 4
December 2016
Pages 516-521

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