Skin Rash and Interstitial Pneumonia Can Be a Fatal Combination: A Rare Case of Anti-Melanoma Differentiation-Associated Gene 5 (MDA5)-Associated Interstitial Lung Disease

  • Giorgia Borio Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Chiara Terracciano Neurology Unit, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Federico Buttafava Intensive Care Unit, IRCCS San Matteo di Pavia, Italy
  • Andrea Vercelli Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Laura Pagani Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Chiara Zanzani Intensive Care Unit, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Alessandra Manicardi Intensive Care Unit, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Andrea Magnacavallo Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy
  • Erika Poggiali Emergency Department, Guglielmo da Saliceto Hospital, Piacenza, Italy

Keywords

MDA5-associated dermatomyositis, MDA5-associated interstitial lung disease, interstitial pneumonia, dyspnoea

Abstract

We report the case of a 62-year-old male patient fully vaccinated for COVID-19, admitted to our emergency room for persistent fever associated with exertional dyspnoea, skin lesions, diffuse myalgias and arthralgias not responsive to broad-spectrum antibiotic and antiviral therapy, who developed a rapidly progressive refractory to treatment interstitial lung disease due to anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, that required mechanical ventilation and ECMO. Here, we highlight the importance of always considering alternative diagnoses, i.e. viral and autoimmune diseases, including anti-MDA5 antibody screening, when dealing with patients with a skin rash, seronegative polyarthralgias and interstitial pneumonia, or acute respiratory distress syndrome of unknown origin.

VIEW THE ENTIRE ARTICLE

References

  • Sato S, Hoshino K, Satoh T, Fujita T, Kawakami Y, Fujita T, et al. 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 2009;60:2193–2200.
  • Gupta R, Kumar S, Gow P, Hsien-Cheng Chang L, Yen L. Anti-MDA5-associated dermatomyositis. Intern Med J 2020;50(4):484–487.
  • Allenbach Y, Uzunhan Y, Toquet S, Leroux G, Gallay L, Marquet A, et al. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: study of 121 cases. Neurology 2020;95(1):e70–e78.
  • Mehta P, Machado PM, Gupta L. Understanding and managing anti-MDA 5 dermatomyositis, including potential COVID-19 mimicry. Rheumatol Int 2021;41(6):1021–1036.
  • Hamaguchi Y, Kuwana M, Hoshino K, Hasegawa M, Kaji K, Matsushita T, et al. Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study. Arch Dermatol 2011;147:391–398.
  • Kurtzman DJB, Vleugels RA. Anti-melanoma differentiation-associated gene 5 (MDA5) dermatomyositis: a concise review with an emphasis on distinctive clinical features. J Am Acad Dermatol 2018;78(4):776–785.
  • Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol 2016;29:662–673.
  • Gupta L, Muhammed H, Naveen R, Kharbanda R, Gangadharan H, Misra D, et al. Insights into the knowledge, attitude and practices for the treatment of idiopathic inflammatory myopathy from a cross-sectional cohort survey of physicians. Rheumatol Int 2020;40(12):2047–2055.
  • Tokiyama K, Tagawa H, Yokota E, Nagasawa K, Kusaba T, Tsuda Y, et al. [Two cases of amyopathic dermatomyositis with fatal rapidly progressive interstitial pneumonitis]. Ryumachi 1990;30(3):204–209; discussion 209–211.
  • Tsuji H, Nakashima R, Hosono Y, Imura Y, Yagita M, Yoshifuji H, et al. Multicenter prospective study of the efficacy and safety of combined immunosuppressive therapy with high-dose glucocorticoid, tacrolimus, and cyclophosphamide in interstitial lung diseases accompanied by anti-melanoma differentiation-associated gene 5-positive dermatomyositis. Arthritis Rheumatol 2020;72(3):488–498.
  • Shirakashi M, Nakashima R, Tsuji H, Tanizawa K, Handa T, Hosono Y, et al. Efficacy of plasma exchange in anti-MDA5-positive dermatomyositis with interstitial lung disease under combined immunosuppressive treatment. Rheumatology (Oxford) 2020;59(11):3284–3292.
  • Mrosak J, Banasiak K, Edelheit B, Lapin C, Tory H, Collins M. Polymyxin-B hemoperfusion as a novel treatment for rapidly progressive interstitial lung disease in a pediatric patient diagnosed with anti-MDA5 juvenile dermatomyositis. J Clin Rheumatol. Epub 2019 Nov 15. doi: 10.1097/RHU.0000000000001191.
  • Hayashi M, Aoki A, Asakawa K, Sakagami T, Kikuchi T, Takada T. Cytokine profiles of amyopathic dermatomyositis with interstitial lung diseases treated with mycophenolate. Respirol Case Rep 2017;5(4):e00235.
  • Mao MM, Xia S, Guo BP, Qian WP, Zheng ZX, Peng XM, et al. Ultra-low dose rituximab as add-on therapy in anti-MDA5-positive patients with polymyositis/dermatomyositis associated ILD. Respir Med 2020;172:105983.
  • Ye S, Chen XX, Lu XY, Wu MF, Deng Y, Huang WQ, et al. Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol 2007;26:1647–1654.
  • Kurasawa K, Arai S, Namiki Y, Tanaka A, Takamura Y, Owada T, et al. Tofacitinib for refractory interstitial lung diseases in anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis. Rheumatology (Oxford) 2018;57:2114–2119.
  • Chen Z, Wang X, Ye S. Tofacitinib in amyopathic dermatomyositis-associated interstitial lung disease. N Engl J Med 2019;381(3):291–293.
  • Li T, Guo L, Chen Z, Gu L, Sun F, Tan X, et al. Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis. Sci Rep 2016;6:33226.
  • Romero-Bueno F, Diaz Del Campo P, Trallero-Araguás E, Ruiz-Rodríguez J, Castellvi I, Rodriguez-Nieto M, et al. Recommendations for the treatment of anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated rapidly progressive interstitial lung disease. Semin Arthritis Rheum 2020;50(4):776–790.
  • Published: 2021-10-18

    Issue: 2021: Vol 8 No 10 (view)

    Section: Articles

    How to cite:
    1.
    Borio G, Terracciano C, Buttafava F, Vercelli A, Pagani L, Zanzani C, Manicardi A, Magnacavallo A, Poggiali E. Skin Rash and Interstitial Pneumonia Can Be a Fatal Combination: A Rare Case of Anti-Melanoma Differentiation-Associated Gene 5 (MDA5)-Associated Interstitial Lung Disease. EJCRIM 2021;8 doi:10.12890/2021_002860.

    Most read articles by the same author(s)