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

  • Yukai WangEmail author
  • Shaoqi ChenEmail author
  • Zhangzhang Lin
  • Jianqun Lin
  • Xuezhen Xie
  • Qisheng Lin
  • Guangzhou Du
  • Xiufeng Huang
  • Marco Matucci-Cerinic
  • Daniel E Furst
Case Based Review


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.


Anti-MDA-5 antibody B-lines Clinically amyopathic dermatomyositis Follow-up Interstitial lung disease KL-6 


Compliance with ethical standards




  1. 1.
    Sato S, Kuwana M (2010) Clinically amyopathic dermatomyositis. Curr Opin Rheumatol 22:639–643CrossRefGoogle Scholar
  2. 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 60:2193–2200CrossRefGoogle Scholar
  3. 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 19:206CrossRefGoogle Scholar
  4. 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.
  5. 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 176:395–402CrossRefGoogle Scholar
  6. 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 18:113–122CrossRefGoogle Scholar
  7. 7.
    Doyle TJ, Dellaripa PF (2017) Lung manifestations in the rheumatic diseases. Chest 152:1283–1295CrossRefGoogle Scholar
  8. 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 123:721–722Google Scholar
  9. 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 2015:107275Google Scholar
  10. 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 34:11–16Google Scholar
  11. 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 72:390–395CrossRefGoogle Scholar
  12. 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 41:1005–1010CrossRefGoogle Scholar
  13. 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 142:965–972CrossRefGoogle Scholar
  14. 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 38:577–591CrossRefGoogle Scholar
  15. 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 37:1244–1251CrossRefGoogle Scholar
  16. 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 109:1207–1212CrossRefGoogle Scholar
  17. 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 24:411–419CrossRefGoogle Scholar
  18. 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 50:3–13CrossRefGoogle Scholar
  19. 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 45:1153–1158CrossRefGoogle Scholar
  20. 20.
    Prasse A, Muller-Quernheim J (2009) Non-invasive biomarkers in pulmonary fibrosis. Respirology 14:788–795CrossRefGoogle Scholar
  21. 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 35:663–666CrossRefGoogle Scholar
  22. 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 9:362–371CrossRefGoogle Scholar
  23. 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 59:257–262CrossRefGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  • Yukai Wang
    • 1
    • 2
    Email author
  • Shaoqi Chen
    • 3
    Email author
  • Zhangzhang Lin
    • 4
  • Jianqun Lin
    • 1
  • Xuezhen Xie
    • 1
  • Qisheng Lin
    • 1
  • Guangzhou Du
    • 5
  • Xiufeng Huang
    • 6
  • Marco Matucci-Cerinic
    • 2
  • Daniel E Furst
    • 7
  1. 1.Department of Rheumatology and ImmunologyShantou Central HospitalShantouChina
  2. 2.Department of Geriatric Medicine, Division of Rheumatology AOUCUniversity of FlorenceFlorenceItaly
  3. 3.Department of UltrasoundThe First Affiliated Hospital of Shantou University Medical CollegeShantouChina
  4. 4.Department of RadiologyJieyang People’s HospitalJieyangChina
  5. 5.Department of RadiologyShantou Central HospitalShantouChina
  6. 6.Department of Respiratory MedicineShantou Central HospitalShantouChina
  7. 7.Division of Rheumatology, Department of MedicineUniversity of California at Los AngelesLos AngelesUSA

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