Skip to main content
SpringerLink
Log in

Utilize lung ultrasound B-lines and KL-6 to monitor anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis-associated interstitial lung disease: a case report and literature review

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

Abstract

In the past decade, lung ultrasound (LUS) B-lines and serum Krebs von den Lungen-6 (KL-6) antigen have been recognized as biomarkers of the connective tissue disease-associated interstitial lung diseases (CTD-ILDs). Robust data have demonstrated that B-lines total numbers and KL-6 levels are correlated with high-resolution computed tomography findings, pulmonary function test, and some clinical parameters in CTD-ILDs. However, limited data are available regarding the use of these two biomarkers to follow CTD-ILDs. Herein, we report a case with anti-melanoma differentiation-associated gene 5 antibody-positive clinically amyopathic dermatomyositis-associated ILD, successfully treated with high-dose methylprednisolone, cyclophosphamide, intravenous immunoglobulin, pirfenidone, and followed using lung ultrasound and KL-6.

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. Sato S, Kuwana M (2010) Clinically amyopathic dermatomyositis. Curr Opin Rheumatol 22:639–643

    Article  PubMed  Google Scholar 

  2. Sato S, Hoshino K, Satoh T, Fujita T, Kawakami Y, Fujita T, Kuwana M (2009) RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: association with rapidly progressive interstitial lung disease. Arthritis Rheum 60:2193–2200

    Article  CAS  PubMed  Google Scholar 

  3. Wang Y, Gargani L, Barskova T, Furst DE, Cerinic MM (2017) Usefulness of lung ultrasound B-lines in connective tissue disease-associated interstitial lung disease: a literature review. Arthritis Res Ther 19:206

    Article  PubMed  PubMed Central  Google Scholar 

  4. Wang Y, Chen S, Lin Z, Du G, Lin J, Lin Q et al (2018) Imaging and serum biomarkers in connective tissue disease-associated interstitial lung diseases: correlation between lung ultrasound B-lines and KL-6 levels. Ann Rheum Dis. https://doi.org/10.1136/annrheumdis-2018-214098

  5. Matsushita T, Mizumaki K, Kano M, Yagi N, Tennichi M, Takeuchi A, Okamoto Y, Hamaguchi Y, Murakami A, Hasegawa M, Kuwana M, Fujimoto M, Takehara K (2017) Antimelanoma differentiation-associated protein 5 antibody level is a novel tool for monitoring disease activity in rapidly progressive interstitial lung disease with dermatomyositis. Br J Dermatol 176:395–402

    Article  CAS  PubMed  Google Scholar 

  6. Barba T, Fort R, Cottin V, Provencher S, Durieu I, Jardel S, Hot A, Reynaud Q, Lega JC (2019) Treatment of idiopathic inflammatory myositis associated interstitial lung disease: a systematic review and meta-analysis. Autoimmun Rev 18:113–122

    Article  PubMed  Google Scholar 

  7. Doyle TJ, Dellaripa PF (2017) Lung manifestations in the rheumatic diseases. Chest 152:1283–1295

    Article  PubMed  PubMed Central  Google Scholar 

  8. Buda N, Kosiak W, Smolenska Z, Zdrojewski Z (2013) Transthoracic lung ultrasound in the monitoring of interstitial lung disease: a case of scleroderma. Pol Arch Med Wewn 123:721–722

    PubMed  Google Scholar 

  9. Laria A, Lurati A, Scarpellini M (2015) Ultrasound in rheumatologic interstitial lung disease: a case report of nonspecific interstitial pneumonia in rheumatoid arthritis. Case Rep Rheumatol 2015:107275

    CAS  PubMed  PubMed Central  Google Scholar 

  10. Song G, Bae SC, Lee YH (2016) Diagnostic accuracy of lung ultrasound for interstitial lung disease in patients with connective tissue diseases: a meta-analysis. Clin Exp Rheumatol 34:11–16

    PubMed  Google Scholar 

  11. Barskova T, Gargani L, Guiducci S, Randone SB, Bruni C, Carnesecchi G, Conforti ML, Porta F, Pignone A, Caramella D, Picano E, Cerinic MM (2013) Lung ultrasound for the screening of interstitial lung disease in very early systemic sclerosis. Ann Rheum Dis 72:390–395

    Article  PubMed  Google Scholar 

  12. Wells G, Beaton DE, Tugwell P, Boers M, Kirwan JR, Bingham CO 3rd et al (2014) Updating the OMERACT filter: discrimination and feasibility. J Rheumatol 41:1005–1010

    Article  PubMed  PubMed Central  Google Scholar 

  13. Reissig A, Copetti R, Mathis G, Mempel C, Schuler A, Zechner P et al (2012) Lung ultrasound in the diagnosis and follow-up of community-acquired pneumonia: a prospective, multicenter, diagnostic accuracy study. Chest 142:965–972

    Article  PubMed  Google Scholar 

  14. Volpicelli G, Elbarbary M, Blaivas M, Lichtenstein DA, Mathis G, Kirkpatrick AW et al (2012) International evidence-based recommendations for point-of-care lung ultrasound. Intensive Care Med 38:577–591

    Article  Google Scholar 

  15. Platz E, Lewis EF, Uno H, Peck J, Pivetta E, Merz AA, Hempel D, Wilson C, Frasure SE, Jhund PS, Cheng S, Solomon SD (2016) Detection and prognostic value of pulmonary congestion by lung ultrasound in ambulatory heart failure patients. Eur Heart J 37:1244–1251

    Article  PubMed  PubMed Central  Google Scholar 

  16. Urbankowska E, Krenke K, Drobczynski L, Korczynski P, Urbankowski T, Krawiec M et al (2015) Lung ultrasound in the diagnosis and monitoring of community acquired pneumonia in children. Respir Med 109:1207–1212

    Article  PubMed  Google Scholar 

  17. Cappelli S, Bellando Randone S, Camiciottoli G, De Paulis A, Guiducci S, Matucci-Cerinic M (2015) Interstitial lung disease in systemic sclerosis: where do we stand? Eur Respir Rev 24:411–419

    Article  PubMed  Google Scholar 

  18. Ishikawa N, Hattori N, Yokoyama A, Kohno N (2012) Utility of KL-6/MUC1 in the clinical management of interstitial lung diseases. Respir Investig 50:3–13

    Article  PubMed  Google Scholar 

  19. Salazar GA, Kuwana M, Wu M, Estrada YMRM, Ying J, Charles J et al (2018) KL-6 but not CCL-18 is a predictor of early progression in systemic sclerosis-related interstitial lung disease. J Rheumatol 45:1153–1158

    Article  PubMed  Google Scholar 

  20. Prasse A, Muller-Quernheim J (2009) Non-invasive biomarkers in pulmonary fibrosis. Respirology 14:788–795

    Article  PubMed  Google Scholar 

  21. Oguz EO, Kucuksahin O, Turgay M, Yildizgoren MT, Ates A, Demir N, Kumbasar OO, Kinikli G, Duzgun N (2016) Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study. Clin Rheumatol 35:663–666

    Article  PubMed  Google Scholar 

  22. Yamakawa H, Hagiwara E, Kitamura H, Yamanaka Y, Ikeda S, Sekine A, Baba T, Okudela K, Iwasawa T, Takemura T, Kuwano K, Ogura T (2017) Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease. J Thorac Dis 9:362–371

    Article  PubMed  PubMed Central  Google Scholar 

  23. Bandoh S, Fujita J, Ohtsuki Y, Ueda Y, Hojo S, Tokuda M et al (2000) Sequential changes of KL-6 in sera of patients with interstitial pneumonia associated with polymyositis/dermatomyositis. Ann Rheum Dis 59:257–262

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Author notes

  1. Yukai Wang and Shaoqi Chen contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Immunology, Shantou Central Hospital, Shantou, Guangdong, China

    Yukai Wang, Jianqun Lin, Xuezhen Xie & Qisheng Lin

  2. Department of Geriatric Medicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy

    Yukai Wang & Marco Matucci-Cerinic

  3. Department of Ultrasound, The First Affiliated Hospital of Shantou University Medical College, Shantou, Guangdong, China

    Shaoqi Chen

  4. Department of Radiology, Jieyang People’s Hospital, Jieyang, Guangdong, China

    Zhangzhang Lin

  5. Department of Radiology, Shantou Central Hospital, Shantou, Guangdong, China

    Guangzhou Du

  6. Department of Respiratory Medicine, Shantou Central Hospital, Shantou, Guangdong, China

    Xiufeng Huang

  7. Division of Rheumatology, Department of Medicine, University of California at Los Angeles, Los Angeles, CA, USA

    Daniel E Furst

Authors
  1. Yukai Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shaoqi Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Zhangzhang Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Jianqun Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Xuezhen Xie
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Qisheng Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Guangzhou Du
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Xiufeng Huang
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Marco Matucci-Cerinic
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Daniel E Furst
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding authors

Correspondence to Yukai Wang or Shaoqi Chen.

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

Wang, Y., Chen, S., Lin, Z. et al. Utilize lung ultrasound B-lines and KL-6 to monitor anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis-associated interstitial lung disease: a case report and literature review. Clin Rheumatol 38, 1433–1436 (2019). https://doi.org/10.1007/s10067-019-04462-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-019-04462-z

Keywords

Search

Navigation