RT Journal Article
SR Electronic
T1 The prognostic factor in patients with myositis-associated interstitial lung disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P718
VO 44
IS Suppl 58
A1 Akira Shiraki
A1 Morihide Ando
A1 Takashi Abe
A1 Harunori Nakashima
A1 Michiko Hibi
A1 Joe Shindo
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P718.abstract
AB Introduction :Anti-aminoacyl-transfer RNA synthetase antibodies (anti-ARS-abs) are most common antibodies in patients with polymyositis (PM) /dermatomyositis (DM). Anti-melanoma differentiation-associated gene 5 antibody (Anti-MDA5-ab) is a marker for rapidly progressive and poor prognostic interstitial lung disease (ILD) with amyopathic DM.Objectives:To evaluate the prognosis markers in patients with ILDs associated to PM/DM.Methods :We reviewed clinical (C-reactive protein, Krebs von den Lungen-6, anti-ARS-abs, anti-MDA5-ab, lymphocytes in bronchial lavage, amyopatic DM) and radiological data of the 51 consecutive patients with ILDs related to PM/DM, retrospectively. Anti-ARS-abs and anti-MDA5-ab were screened out by immunoprecipitation techniques in Department of Dermatology, Kanazawa University and ELISA method in Department of Dermatology, Nagoya University.Results :Median age was 61 years old and male gender was 25%. 23 patients had anti-ARS-abs and 9 patients had anti-MDA5 ab. 19 patients had neither of them. 6 patients (26%) with anti-ARS-abs, 5 (56%) with anti-MDA5-ab and 5 (26%) with double negative died in follow up period (45 months, median). C-reactive protein (CRP) > 1.0 (p=0.031, OR 1.91) was the only significant factor between overall survivors and non-survivors. On the other hand, anti-ARS-abs positive (p=0.031, OR 0.54) and anti-MDA5-ab positive (p=0.005, OR 2.04) were significant factors between 1 year survivors and non-survivors. Amyopathic DM was not risk factor on both long and short-term survivals.Conclusion:Anti-MDA5-ab was a risk factor on short-term survivals and CRP value was related to long-term prognosis in this cohort.