Skip to main content

Advertisement

SpringerLink
Log in

Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain

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

Abstract

Objectives

This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated.

Methods

Thirty-five consecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients’ conditions.

Results

Patients and controls had similar age (12.6 ± 0.7 vs.12.5 ± 0.6; p = 0.97) and gender (11F:24M vs.11F:24M; p = 1.0). Median of JDM duration was 4.6 (0.04–17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (≥ 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [−22(−17.2 to −27.9) % vs. −23(−20.8 to −27.4) %; p = 0.028)] and circumferential −23.9 ± 2.8% vs. −26.7 ± 2.9%; p = 0.0002) strain. Lower longitudinal strain was associated with DAS >3 −19.9(−17.2 to −26.5)% vs. −22.1−18.9 to −27.9)%; p = 0.046], MDI extent > 0 [−19(−17.2 to −22.5)% vs. −22.1−19.2 to −27.9)%; p = 0.0008], MDI severity > 0 [−19(−17.2 to −22.1)% vs. −22.3(−20.3 to −27.9)%; p = 0.0001] and calcinosis[−20.6(−17.2 to −23)% vs. −22.3(−20.3 to −27.9)%; p = 0.03]. Lower circumferential strain was associated with MDI extent > 0 (−22.1 ± 3.87% vs. −24.4 ± 2.3%; p = 0.039), MDI severity > 0 (−21.7 ± 3% vs. 24.7 ± 2.3%; p = 0.004) and calcinosis (−22.5 ± 3.3% vs. −24.8 ± 2.1%; p = 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r = −0.44; p = 0.009) and methotrexate (r = −0.33; p = 0.0008).

Conclusions

LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage.

Key Points:

• Serious cardiac involvement is rare but has been associated with death in juvenile dermatomyositis.

• Two-dimensional speckle tracking stands out for the identification of subclinical myocardial compromise in juvenile dermatomyositis.

• Longitudinal strain impairment is associated with disease activity and cumulative damage, whereas circumferential strain impairment is associated exclusively with cumulative damage.

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
Fig 2

Similar content being viewed by others

References

  1. Rider LG, Lindsley CB, Miller FW (2016) Juvenile dermatomyositis. In: Petty RE, Laxer RM, Lindsley CB, Wedderburn LR (eds) Textbook of Pediatric Rheumatology, 7th edn. Elsevier, Philadelphia, pp 351–383

    Chapter  Google Scholar 

  2. Schwartz T, Sanner H, Gjesdal O, Flato B, Sjaastad I (2014) In juvenile dermatomyositis, cardiac systolic dysfunction is present after long-term follow-up and is predicted by sustained early skin activity. Ann Rheum Dis 73:1805–1810. https://doi.org/10.1136/annrheumdis-2013-203279

    Article  PubMed  Google Scholar 

  3. Caforio ALP, Adler Y, Agostini C, Allanore Y, Anastasakis A, Arad M, Böhm M, Charron P, Elliott PM, Eriksson U, Felix SB, Garcia-Pavia P, Hachulla E, Heymans S, Imazio M, Klingel K, Marcolongo R, Matucci Cerinic M, Pantazis A, Plein S, Poli V, Rigopoulos A, Seferovic P, Shoenfeld Y, Zamorano JL, Linhart A (2017) Diagnosis and management of myocardial involvement in systemic immune-mediated diseases: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease. Eur Heart J 38(35):2649–2662. https://doi.org/10.1093/eurheartj/ehx321

    Article  CAS  PubMed  Google Scholar 

  4. Leal GN, Silva KF, Lianza AC, Giacomin MF, Andrade JL, Kozu K, Bonfá E, Silva CA (2016) Subclinical left ventricular dysfunction in childhood-onset systemic lupus erythematosus: a two-dimensional speckle-tracking echocardiographic study. Scand J Rheumatol 45:202–229. https://doi.org/10.3109/03009742.2015.1063686

    Article  CAS  PubMed  Google Scholar 

  5. Leal GN, Silva KF, França CM, Lianza AC, Andrade JL, Campos LM, Bonfá E, Silva CA (2015) Subclinical right ventricle systolic dysfunction in childhood-onset systemic lupus erythematosus: insights from two-dimensional speckle-tracking echocardiography. Lupus 24:613–620. https://doi.org/10.1177/0961203314563135

    Article  CAS  PubMed  Google Scholar 

  6. Guerra F, Gelardi C, Capucci A, Gabrielli A, Danieli MG (2017) Subclinical Cardiac Dysfunction in polymyositis and dermatomyositis: a speckle-tracking case-control study. J Rheumatol 44:815–821. https://doi.org/10.3899/jrheum.161311

    Article  CAS  PubMed  Google Scholar 

  7. Bohan A, Peter JB (1975) Polymyositis and dermatomyositis. N Engl J Med 13:344–347. https://doi.org/10.1056/NEJM197502132920706

    Article  Google Scholar 

  8. Dulcan M (1994) Nomenclature and criteria for diagnosis of diseases of the heart and great vessels, 9th edn. Little, Brown & Co, Boston, pp 253–256

    Google Scholar 

  9. Huber AM, Feldman BM, Rennebohm RM, Hicks JE, Lindsley CB, Perez MD et al (2004) Validation and clinical significance of the Childhood Myositis Assessment Scale for assessment of muscle function in the juvenile idiopathic inflammatory myopathies. Arthritis Rheum 50:1595–1603. https://doi.org/10.1002/art.20179

    Article  PubMed  Google Scholar 

  10. Lazarevic D, Pistorio A, Palmisani E, Miettunen P, Ravelli A, Pilkington C et al (2013) The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis. Ann Rheum Dis 72:686–693. https://doi.org/10.1136/annrheumdis-2012-201483

    Article  CAS  PubMed  Google Scholar 

  11. Rider LG, Werth VP, Huber AM, Alexanderson H, Rao AP, Ruperto N, Herbelin L, Barohn R, Isenberg D, Miller FW (2011) Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: Physician and Patient/Parent Global Activity, Manual Muscle Testing (MMT), Health Assessment Questionnaire (HAQ)/Childhood Health Assessment Questionnaire (C-HAQ), Childhood Myositis Assessment Scale (CMAS), Myositis Disease Activity Assessment Tool (MDAAT), Disease Activity Score (DAS), Short Form 36 (SF-36), Child Health Questionnaire (CHQ), physician global damage, Myositis Damage Index (MDI), Quantitative Muscle Testing (QMT), Myositis Functional Index-2 (FI-2), Myositis Activities Profile (MAP), Inclusion Body Myositis Functional Rating Scale (IBMFRS), Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), Cutaneous Assessment Tool (CAT), Dermatomyositis Skin Severity Index (DSSI), Skindex, and Dermatology Life Quality Index (DLQI). Arthritis Care Res 63(Suppl 11):S118–S157. https://doi.org/10.1002/acr.20532

    Article  Google Scholar 

  12. Apaz MT, Saad-Magalhães C, Pistorio A, Ravelli A, Sato JO, Marcantoni MB, Meiorin S, Filocamo G, Pilkington C, Maillard S, Al-Mayouf S, Prahalad S, Fasth A, Joos R, Schikler K, Mozolova D, Landgraf JM, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (2009) Health-related quality of life of patients with juvenile dermatomyositis: results from the paediatric rheumatology international trials organisation multinational quality of life cohort study. Arthritis Rheum 6:509–517. https://doi.org/10.1002/art.24343

    Article  Google Scholar 

  13. Klatchoian DA, Len CA, Terreri MT, Silva M, Itamoto C, Ciconelli RM, Varni JW, Hilário MO (2008) Quality of life of children and adolescents from São Paulo: reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory version 4.0 Generic Core Scales. J Pediatr 84:308–315. https://doi.org/10.2223/JPED.1788

    Article  Google Scholar 

  14. Kountz-Edwards S, Aoki C, Gannon C, Gomez R, Cordova M, Packman W (2017) The family impact of caring for a child with juvenile dermatomyositis. Chronic Illn 13:262–274. https://doi.org/10.1177/1742395317690034

    Article  PubMed  Google Scholar 

  15. Haycock GB, Schwartz GJ, Wisotsky DH (1978) Geometric method for measuring body surface area: a height-weight formula validated in infants, children, and adults. J Pediatr 93:62–66. https://doi.org/10.1016/s0022-3476(78)80601-5

    Article  CAS  PubMed  Google Scholar 

  16. Lopez L, Colan SD, Frommelt PC, Ensing GJ, Kendall K, Younoszai AK, Lai WW, Geva T (2010) Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the pediatric measurements writing group of the American society of echocardiography pediatric and congenital heart disease council. J Am Soc Echocardiogr 23:465–495. https://doi.org/10.1016/j.echo.2010.03.019

    Article  PubMed  Google Scholar 

  17. Mor-Avi V, Mor-Avi V, Lang RM, Badano LP, Belohlavek M, Cardim NM, Derumeaux G, Galderisi M, Marwick T, Nagueh SF, Sengupta PP, Sicari R, Smiseth OA, Smulevitz B, Takeuchi M, Thomas JD, Vannan M, Voigt JU, Zamorano JL (2011) Current and Evolving Echocardiographic Techniques for the Quantitative Evaluation of Cardiac Mechanics: ASE/EAE Consensus Statement on Methodology and Indications Endorsed by the Japanese Society of Echocardiography. J Am Soc Echocardiogr 24:277–313. https://doi.org/10.1016/j.echo.2011.01.015

    Article  PubMed  Google Scholar 

  18. Levy PT, Machefsky A, Sanchez AA, Patel MD, Rogal S, Fowler S, Yaeger L, Hardi A, Holland MR, Hamvas A, Singh GK (2016) Reference ranges of left ventricular strain measures by two-dimensional speckle-tracking echocardiography in children: a systematic review and meta-analysis. J Am Soc Echocardiogr 29:209–225. https://doi.org/10.1016/j.echo.2015.11.016

    Article  PubMed  Google Scholar 

  19. Eidem BW, McMahon CJ, Cohen RR, Wu J, Finkelshteyn I, Kovalchin JP, Ayres NA, Bezold LI, O'Brian Smith E, Pignatelli RH (2004) Impact of cardiac growth on Doppler tissue imaging velocities: a study in healthy children. J Am Soc Echocardiogr 17:212–221. https://doi.org/10.1016/j.echo.2003.12.005

  20. Schawartz T, Sanner H, Husebye T, Flato B, Sjaastad I (2011) Cardiac dysfunction in juvenile dermatomyositis: a case-control study. Ann Rheum Dis 70:766–771. https://doi.org/10.1136/ard.2010.137968

    Article  Google Scholar 

  21. Gupta R, Wayangankar SA, Targoff IN, Hennebry TA (2011) Clinical cardiac involvement in idiopathic inflammatory myopathies: a systematic review. Int J Cardiol 148:261–270. https://doi.org/10.1016/j.ijcard.2010.08.013

    Article  PubMed  Google Scholar 

  22. Leal GN (2019) Applications of the Myocardial Strain Study using Two-Dimensional Speckle Tracking in Pediatrics. Arq Bras Cardiol: Imagem Cardiovasc 32:29–33. https://doi.org/10.5935/2318-8219.20190008

    Article  Google Scholar 

  23. Mavrogeni S, Douskou M, Manoussakis MN (2011) Contrast-Enhanced CMR Imaging Reveals Myocardial Involvement in Idiopathic Inflammatory Myopathy Without Cardiac Manifestations. JACC Cardiovasc Imaging 4:1324–1325. https://doi.org/10.1016/j.jcmg.2011.05.009

    Article  PubMed  Google Scholar 

  24. Schwartz T, Diederichsen LP, Lundberg IE, Sjaastad I, Sanner H (2016) Cardiac involvement in adult and juvenile idiopathic inflammatory myopathies. RMD Open 2016:e000291. https://doi.org/10.1136/rmdopen-2016-000291

    Article  Google Scholar 

  25. Wienke J, Deakin CT, Wedderburn LR, van Wijk F, van Royen-Kerkhof A (2018) Systemic and Tissue Inflammation in Juvenile Dermatomyositis: From Pathogenesis to the Quest for Monitoring Tools. Front Immunol 9:2951. https://doi.org/10.3389/fimmu.2018.02951

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Rosa Neto NS, Goldenstein-Schainberg C (2010) Juvenile dermatomyositis: review and update of the pathogenesis and treatment. Rev Bras Reumatol 50:299–312

    Article  PubMed  Google Scholar 

  27. Bottai M, Tjärnlund A, Santoni G, The International Myositis Classification Criteria Project consortium, the Euromyositis register and the Juvenile Dermatomyositis Cohort Biomarker Study and Repository (JDRG) (UK and Ireland) et al (2017) EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: a methodology report. RMD Open 3:e000507. https://doi.org/10.1136/rmdopen-2017-000507

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Funding

This work was supported by grant from Fundação do Amparo a Pesquisa do Estado de São Paulo (FAPESP) (#2015/03756-4 to CAS), Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPQ #303422/2015-7 to CAS), and by Núcleo de Apoio à Pesquisa “ Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS.

Author information

Authors and Affiliations

  1. Pediatric Echocardiography Laboratory, Instituto da Criança e do Adolescente do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Maria de Fátima Rodrigues Diniz, Alessandro Cavalcanti Lianza, Karen Saori Shiraishi Sawamura, Carolina Rocha Brito Menezes & Gabriela Nunes Leal

  2. Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Katia Tomie Kozu, Adriana Maluf Elias, Juliana Caires de Oliveira Achili Ferreira & Clovis Artur Silva

Authors
  1. Maria de Fátima Rodrigues Diniz
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Katia Tomie Kozu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Adriana Maluf Elias
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Alessandro Cavalcanti Lianza
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Karen Saori Shiraishi Sawamura
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Carolina Rocha Brito Menezes
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Juliana Caires de Oliveira Achili Ferreira
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Clovis Artur Silva
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Gabriela Nunes Leal
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maria de Fátima Rodrigues Diniz.

Ethics declarations

Disclosures

None.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Diniz, M.d.R., Kozu, K.T., Elias, A.M. et al. Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain. Clin Rheumatol 40, 1497–1505 (2021). https://doi.org/10.1007/s10067-020-05418-4

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-020-05418-4

Keywords

Search

Navigation