Abstract
BackgroundInterstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM-ILD) is often refractory and rapidly progressive. Although the anti-melanoma differentiation-associated gene 5 (anti-MDA-5) antibody is associated with rapidly progressive ILD (RP-ILD), differences in clinical features and prognosis of anti-MDA-5 antibody-positive and -negative CADM-ILD remain unclear.MethodsTo clarify the differences in the clinical features and prognosis between anti-MDA-5 antibody-positive and -negative cases, we retrospectively reviewed the medical records of patients diagnosed with CADM-ILD with and without anti-MDA-5 antibody at Kurashiki Central Hospital from January 2005 to September 2014.ResultsAnti-MDA-5 antibody was found in 10 of 16 patients (63 %). The levels of Krebs von den Lungen-6 (KL-6) and surfactant protein D (SP-D) at the first visit were significantly lower in positive patients than in negative patients, whereas the levels of aspartate aminotransferase (AST), γ-glutamyl transpeptidase (γ-GTP), and the CD4+/CD8+ ratio in the bronchoalveolar lavage (BAL) fluid were significantly higher in positive patients than negative patients. Subpleural ground-glass opacity (GGO) or irregular linear opacity was predominant in positive patients. Peribronchovascular consolidation was predominant in negative patients. Positive patients had significantly lower survival rates than negative patients, with all six fatal cases occurring in positive patients who died of refractory ILD within 92 days from the first visit despite intensive treatment.ConclusionsThere are clear differences in the clinical features and prognosis of anti-MDA-5 antibody-positive and -negative CADM-ILD. Low serum KL-6 and SP-D levels, high serum AST and γ-GTP levels, high CD4+/CD8+ ratio in BAL fluid, and predominance of subpleural GGO or irregular linear opacity in HRCT may help to discriminate anti-MDA-5 antibody-positive CADM-ILD with poor prognosis.
Highlights
Interstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM-ILD) is often refractory and rapidly progressive
ILD associated with CADM (CADM-ILD) is often refractory and rapidly progressive [1,2,3], resulting in respiratory failure with a 6-month survival rate of 40.8–54.5 % [3,4,5]
As melanoma differentiation-associated gene 5 (MDA-5) plays the critical role in the innate immune defense against viruses, one hypothesis is that the production of anti–MDA-5 antibodies is a secondary phenomenon during virus infection that is associated with the onset of CADM and rapidly progressive ILD (RP-ILD)
Summary
Interstitial lung disease (ILD) associated with clinically amyopathic dermatomyositis (CADM-ILD) is often refractory and rapidly progressive. The anti-melanoma differentiation-associated gene 5 (anti-MDA-5) antibody is associated with rapidly progressive ILD (RP-ILD), differences in clinical features and prognosis of anti-MDA-5 antibody-positive and -negative CADM-ILD remain unclear. Interstitial lung disease (ILD) is the most common internal organ manifestation that affects the prognosis of clinically amyopathic dermatomyositis (CADM), as well as polymyositis (PM) and dermatomyositis (DM). Among patients with CADM, anti-melanoma differentiation-associated gene 5 (MDA-5) antibodies are strongly associated with the development of rapidly progressive ILD (RP-ILD) [8,9,10]. As MDA-5 plays the critical role in the innate immune defense against viruses, one hypothesis is that the production of anti–MDA-5 antibodies is a secondary phenomenon during virus infection that is associated with the onset of CADM and RP-ILD. To our knowledge, no previous study has revealed whether the presence or absence of the antiMDA-5 antibody affects the clinical manifestation of CADM-ILD
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