Abstract
Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, showing a high incidence of lung involvements. The aim of this study is to identify risk factors, other than melanoma differentiation-associated protein (MDA)-5, for developing rapidly progressive-interstitial lung disease (RP-ILD) in patients with CADM. Forty CADM patients, in whom 11 patients developed RP-ILD, were enrolled. Clinical features and laboratory findings were compared between the patients with and without RP-ILD. We found that skin ulceration, CRP, serum ferritin, anti-MDA5 Ab, and lymphocytopenia were significantly associated with ILD. Multivariate logistic regression analysis indicated that anti-MDA5 Ab+, elevated CRP, and decreased counts of lymphocyte were independent risk factors for RP-ILD, which can provide a precise predict for RP-ILD in CADM patients. When anti-MDA5 Ab+ was removed from the multivariate regression model, using skin ulcerations, elevated serum ferritin and decreased counts of lymphocyte can also precisely predict RP-ILD. Except for MDA-5, more commonly available clinical characteristics, such as skin ulcerations, serum ferritin, and count of lymphocyte may also help to predict prognosis in CADM.
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References
Sontheimer RD (2002) Would a new name hasten the acceptance of amyopathic dermatomyositis (dermatomyositis siné myositis) as a distinctive subset within the idiopathic inflammatory dermatomyopathies spectrum of clinical illness? J Am Acad Dermatol 46:626–636
Gerami P, Schope JM, McDonald L et al (2006) A systematic review of adult-onset clinically amyopathic dermatomyositis (dermatomyositis siné myositis): a missing link within the spectrum of the idiopathic inflammatory myopathies. J Am Acad Dermatol 54:597–613
Ghazi E, Sontheimer RD, Werth VP (2013) The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum. Clin Exp Rheumatol 31:128–134
Ye S, Chen XX, Lu XY et al (2007) Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study. Clin Rheumatol 26:1647–1654
Fathi M, Lundberg IE (2005) Interstitial lung disease in polymyositis and dermatomyositis. Curr Opin Rheumatol 17:701–706
Kameda H, Nagasawa H, Ogawa H et al (2005) Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 32:1719–1726
Sato S, Hirakata M, Kuwana M et al (2005) Autoantibodies to a 140-kd polypeptide, CADM-140, in Japanese patients with clinically amyopathic dermatomyositis. Arthritis Rheum 52:1571–1576
Sato S, Hoshino K, Satoh T et al (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
Cao H, Pan M, Kang Y et al (2012) Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti-melanoma differentiation-associated gene 5 antibody. Arthritis Care Res (Hoboken) 64:1602–1610
Koga T, Fujikawa K, Horai Y et al (2012) The diagnostic utility of anti-melanoma differentiation-associated gene 5 antibody testing for predicting the prognosis of Japanese patients with DM. Rheumatol (United Kingdom) 51:1278–1284
Hoogendijk JE, Amato AA, Lecky BR, et al. (2004) 119th ENMC international workshop: Trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis. Neuromuscul Disord 14:337–345
American Thoracic Society (2000) Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 161:646–664
Suda T, Fujisawa T, Enomoto N et al (2006) Interstitial lung diseases associated with amyopathic dermatomyositis. Eur Respir J Off J Eur Soc Clin Respir Physiol 28:1005–1012
Ishigaki K, Maruyama J, Hagino N et al (2013) Skin ulcer is a predictive and prognostic factor of acute or subacute interstitial lung disease in dermatomyositis. Rheumatol Int 33:2381–2389
Gono T, Kawaguchi Y, Ozeki E et al (2011) Serum ferritin correlates with activity of anti-MDA5 antibody-associated acute interstitial lung disease as a complication of dermatomyositis. Mod Rheumatol 21:223–227
Gono T, Kawaguchi Y, Hara M et al (2010) Increased ferritin predicts development and severity of acute interstitial lung disease as a complication of dermatomyositis. Rheumatology 49:1354–1360
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The work is supported by National Nature Science Foundation of China of under Grant (No. 81271324) and Liaoning Province Nature Science Funds (201202250) to PT Yang.
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The whole research was approved by the local ethic commission of the First Affiliated Hospital of China Medical University.
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Y. Xu and C. S. Yang contributed equally to this work.
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Xu, Y., Yang, C.S., Li, Y.J. et al. Predictive factors of rapidly progressive-interstitial lung disease in patients with clinically amyopathic dermatomyositis. Clin Rheumatol 35, 113–116 (2016). https://doi.org/10.1007/s10067-015-3139-z
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DOI: https://doi.org/10.1007/s10067-015-3139-z