Clinical manifestation and prognostic factor in anti-melanoma differentiation-associated gene 5 antibody-associated interstitial lung disease as a complication of dermatomyositis.
OBJECTIVE
The aim of this study is to evaluate the clinical manifestation and prognostic factors of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-associated interstitial lung disease (ILD) with DM.


METHODS
Fourteen patients who presented with anti-MDA5 antibody and 10 patients with anti-aminoacyl-tRNA synthetase (ARS) antibody were enrolled. All patients were diagnosed as having DM with ILD. Clinical manifestations in the patients with anti-MDA5 antibody were compared with those in the patients with anti-ARS antibody.


RESULTS
The frequencies of acute/subacute interstitial pneumonia (A/SIP) and fatal outcome were significantly higher in the subset with anti-MDA5 antibody. The creatine kinase (CK) value was significantly lower and the gamma-glutamyl transpeptidase and ferritin values were significantly higher in the subset with anti-MDA5 antibody. Significant correlations were found between PaO(2)/F(i)O(2) and ferritin (r(s) = -0.59, P = 0.035), alveolar-arterial oxygen difference (A-aDO(2)) and KL-6 (r(s) = 0.73, P = 0.016) and A-aDO(2) and ferritin (r(s) = 0.66, P = 0.013) in the subset with anti-MDA5 antibody. The most significant prognostic factor was ferritin. The cumulative survival rate was significantly lower (P < 0.0001) in the subset with ferritin >or=1600 ng/ml than that in the subset with ferritin <1600 ng/ml in anti-MDA5 antibody-associated ILD.


CONCLUSIONS
Both serum ferritin and anti-MDA5 antibody are powerful indicators for the early diagnosis of A/SIP with DM. Ferritin also predicts disease severity and prognosis for patients with anti-MDA5 antibody. Intensive treatment should be administered to cases that have anti-MDA5 antibody-associated ILD with DM showing hyperferritinaemia, especially if the ferritin level is >or=1600 ng/ml.
Gono
Takahisa
T
Institute of Rheumatology, Tokyo Women's Medical University, 10-22 Kawada-cho, Shinjuku-Ku, Tokyo 162-0054, Japan.
Kawaguchi
Yasushi
Y
Satoh
Takashi
T
Kuwana
Masataka
M
Katsumata
Yasuhiro
Y
Takagi
Kae
K
Masuda
Ikuko
I
Tochimoto
Akiko
A
Baba
Sayumi
S
Okamoto
Yuko
Y
Ota
Yuko
Y
Yamanaka
Hisashi
H
D000328:Adult; D001323:Autoantibodies / Q000235:genetics / Q000276:immunology*; D015415:Biomarkers; D053487:DEAD-box RNA Helicases / Q000235:genetics / Q000276:immunology*; D003882:Dermatomyositis / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005293:Ferritins / Q000276:immunology*; D006801:Humans; D000072640:Interferon-Induced Helicase, IFIH1; D017563:Lung Diseases, Interstitial / Q000235:genetics / Q000276:immunology* / Q000503:physiopathology; D008262:Macrophage Activation / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013223:Statistics as Topic
D001323:Autoantibodies; D015415:Biomarkers; D005293:Ferritins; C452815:IFIH1 protein, human; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1