Rheumatology International

, Volume 39, Issue 2, pp 293–300 | Cite as

Evaluation of six-minute walk test in juvenile systemic sclerosis

  • Oya Koker
  • Amra Adrovic
  • Sezgin Sahin
  • Mehmet Yildiz
  • Kenan Barut
  • Rukiye Eker Omeroglu
  • Ozgur KasapcopurEmail author
Observational Research


The objective is to evaluate the walking distance and oxygen desaturation during the six-minute walk test and to establish correlations between the test results and other clinical findings so to assess the reliability of the test for evaluation of children with juvenile systemic sclerosis (jSSc). A total of 25 jSSc, 27 juvenile systemic lupus erythematosus (jSLE), and 30 healthy controls were included. The test is conducted according to the guidelines recommended by the American Thoracic Society, standardized in 2002. Median values of walking distances were 470 (415–580) m in jSSc; 518 (376–618) m in jSLE; and 562 (493.5–618) m in healthy controls. jSSc patients walked significantly less distance comparing to controls (p < 0.001). jSSc patients with lung involvement walked less than those without lung involvement [463.2 (418–565) m vs. 491.5 (415–580) m], but without a significant difference (p = 0.82). The frequency of lower extremity pain during and after the test was significantly higher in the jSSc cohort compared to both control groups (p = 0.001). Patients with myalgia were found to walk less than those without myalgia [446.5 (415–538) m vs. 493.5 (428–580) m] (p = 0.04). Patients with jSSc have limited walking distances. Despite the decreased walking distance among jSSc patients with ILD and/or PAH, the limited number of patients makes the results inappropriate for interpretation. Low extremity pain influences the walking capacity of jSSc patients.


Pediatric systemic sclerosis Six-minute walk test Pulmonary arterial hypertension Interstitial lung disease 


Author contributions

OK contributed to the conception and design of the study, interpretation of the data, OK and AA contributed to the data collection, data analysis and drafting article, KB and REO contributed to the acquisition and evaluation of the data, MY and SS participated in data collection and performing the statistical analysis. All authors read and approved the manuscript.


This review was not funded by any person or corporation.

Compliance with ethical standards

Conflict of interest

Author O.K, Author A.A, Author S.S, Author M.Y, Author K.B, Author R.E.O, and Author O.K declare that they have no conflict of interest including specific financial interests, relationships, and/or affiliations relevant to the subject matter or materials.

Ethical approval

Approval was obtained for the study from the Ethics Committee of the Cerrahpasa Medical School (approved: 08/02/2018-53142). Informed consent was obtained from all individual participants included in the study. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.


  1. 1.
    Barsotti S, Bruni C, Orlandi M, Della Rossa A, Marasco E, Codullo V, Guiducci S (2017) One year in review 2017: systemic sclerosis. Clin Exp Rheumatol 35(Suppl 106):3–20Google Scholar
  2. 2.
    Torok KS (2012) Pediatric scleroderma: systemic or localized forms. Pediatr Clin N Am 59(2):381–405. CrossRefGoogle Scholar
  3. 3.
    Adrovic A, Sahin S, Barut K, Kasapcopur O (2018) Juvenile scleroderma-what has changed in the meantime? Curr Rheumatol Rev. Google Scholar
  4. 4.
    Li AM, Yin J, Yu CC, Tsang T, So HK, Wong E, Chan D, Hon EK, Sung R (2005) The six-minute walk test in healthy children: reliability and validity. Eur Respir J 25(6):1057–1060. CrossRefGoogle Scholar
  5. 5.
    Impens AJ, Wangkaew S, Seibold JR (2008) The 6-minute walk test in scleroderma—how measuring everything measures nothing. Rheumatology (Oxford, England) 47(Suppl 5):v68–v69. CrossRefGoogle Scholar
  6. 6.
    Zulian F, Woo P, Athreya BH, Laxer RM, Medsger TA Jr, Lehman TJ, Cerinic MM, Martini G, Ravelli A, Russo R, Cuttica R, de Oliveira SK, Denton CP, Cozzi F, Foeldvari I, Ruperto N (2007) The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum 57(2):203–212. CrossRefGoogle Scholar
  7. 7.
    Clements P, Lachenbruch P, Siebold J, White B, Weiner S, Martin R, Weinstein A, Weisman M, Mayes M, Collier D et al (1995) Inter and intraobserver variability of total skin thickness score (modified Rodnan TSS) in systemic sclerosis. J Rheumatol 22(7):1281–1285Google Scholar
  8. 8.
    La Torre F, Martini G, Russo R, Katsicas MM, Corona F, Calcagno G, Falcini F, Vittadello F, Zulian F (2012) A preliminary disease severity score for juvenile systemic sclerosis. Arthritis Rheum 64(12):4143–4150. CrossRefGoogle Scholar
  9. 9.
    ATS Committee on Proficiency Standards for Clinical Pulmonary Function Laboratories (2002) ATS statement: guidelines for the six-minute walk test. Am J Respir Crit Care Med 166:111–117. CrossRefGoogle Scholar
  10. 10.
    Deuschle K, Weinert K, Becker MO, Backhaus M, Huscher D, Riemekasten G (2011) Six-minute walk distance as a marker for disability and complaints in patients with systemic sclerosis. Clin Exp Rheumatol 29(2 Suppl 65):S53–S59Google Scholar
  11. 11.
    Vandecasteele E, Thevissen K, Melsens K, De Keyser F, De Pauw M, Deschepper E, Decuman S, Piette Y, Brusselle G, Smith V (2017) Six-minute walk test in or out in evaluation of systemic sclerosis patients? Clin Exp Rheumatol 35 Suppl 106(4):122–129Google Scholar
  12. 12.
    Vandecasteele E, De Pauw M, De Keyser F, Decuman S, Deschepper E, Piette Y, Brusselle G, Smith V (2016) Six-minute walk test in systemic sclerosis: a systematic review and meta-analysis. Int J Cardiol 212:265–273. CrossRefGoogle Scholar
  13. 13.
    Adrovic A, Sahin S, Barut K, Kasapcopur O (2018) Juvenile scleroderma referral center experience. Arch Rheumatol 33:344–351CrossRefGoogle Scholar
  14. 14.
    Adrovic A, Oztunc F, Barut K, Koka A, Gojak R, Sahin S, Demir T, Kasapcopur O (2015) The frequency of pulmonary hypertension in patients with juvenile scleroderma. Bosn J Basic Med Sci 15(4):30–35. CrossRefGoogle Scholar
  15. 15.
    Martini G, Foeldvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, Lehman TJ, de Oliveira SKF, Susic G, Lyskina G (2006) Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. Arthritis Rheum 54(12):3971–3978CrossRefGoogle Scholar
  16. 16.
    Lammers AE, Munnery E, Hislop AA, Haworth SG (2010) Heart rate variability predicts outcome in children with pulmonary arterial hypertension. Int J Cardiol 142(2):159–165. CrossRefGoogle Scholar
  17. 17.
    Douwes JM, Hegeman AK, van der Krieke MB, Roofthooft MT, Hillege HL, Berger RM (2016) Six-minute walking distance and decrease in oxygen saturation during the six-minute walk test in pediatric pulmonary arterial hypertension. Int J Cardiol 202:34–39. CrossRefGoogle Scholar
  18. 18.
    Garin MC, Highland KB, Silver RM, Strange C (2009) Limitations to the 6-minute walk test in interstitial lung disease and pulmonary hypertension in scleroderma. J Rheumatol 36(2):330–336. CrossRefGoogle Scholar
  19. 19.
    Lima TR, Guimaraes FS, Silva LA, Silva DP, Menezes SL, Lopes AJ (2015) Relationship between functional capacity, joint mobility and pulmonary function in patients with systemic sclerosis. J Bodyw Mov Ther 19(1):17–24. CrossRefGoogle Scholar
  20. 20.
    Peytrignet S, Denton CP, Lunt M, Hesselstrand R, Mouthon L, Silman A, Pan X, Brown E, Czirjak L, Distler JHW, Distler O, Fligelstone K, Gregory WJ, Ochiel R, Vonk M, Ancuta C, Ong VH, Farge D, Hudson M, Matucci-Cerinic M, Balbir-Gurman A, Midtvedt O, Jordan AC, Stevens W, Moinzadeh P, Hall FC, Agard C, Anderson ME, Diot E, Madhok R, Akil M, Buch MH, Chung L, Damjanov N, Gunawardena H, Lanyon P, Ahmad Y, Chakravarty K, Jacobsen S, MacGregor AJ, McHugh N, Muller-Ladner U, Riemekasten G, Becker M, Roddy J, Carreira PE, Fauchais AL, Hachulla E, Hamilton J, Inanc M, McLaren JS, van Laar JM, Pathare S, Proudman S, Rudin A, Sahhar J, Coppere B, Serratrice C, Sheeran T, Veale DJ, Grange C, Trad GS, Herrick AL (2018) Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study. Rheumatology (Oxford, England) 57(2):370–381. CrossRefGoogle Scholar
  21. 21.
    Weisman IM, Zeballos RJ (1994) An integrated approach to the interpretation of cardiopulmonary exercise testing. Clin Chest Med 15(2):421–445Google Scholar
  22. 22.
    Nielsen HB, Madsen P, Svendsen LB, Roach RC, Secher NH (1998) The influence of PaO2, pH and SaO2 on maximal oxygen uptake. Acta Physiol Scand 164(1):87–89. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatric Rheumatology, Istanbul Faculty of MedicineIstanbul UniversityIstanbulTurkey
  2. 2.Department of Pediatric Rheumatology, Cerrahpasa Medical SchoolIstanbul University-CerrahpasaIstanbulTurkey

Personalised recommendations