PT  - JOURNAL ARTICLE
AU  - Hajime Sasano
AU  - Hideya Kitamura
AU  - Tomohisa Baba
AU  - Yasunori Enomoto
AU  - Norikazu Matsuo
AU  - Eri Hagiwara
AU  - Miyako Nakagawa
AU  - Etsuko Iwata
AU  - Koji Okudera
AU  - Shinji Sato
AU  - Yasuo Suzuki
AU  - Tae Iwasawa
AU  - Tamiko Takemura
AU  - Takashi Ogura
TI  - The clinical features of 13 patients with interstitial pneumonia with positive anti-glycyl-tRNA synthetase autoantibodies
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P726
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P726.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P726.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Objectives: Anti-aminoacyl-tRNA synthetase autoantibodies are highly prevalent in patients with polymyositis/dermatomyositis (PM/DM) particularly in those complicated with interstitial pneumonia (IP). The clinical characteristics in positive anti-glycyl-tRNA synthetase (anti-EJ) autoantibody patients with IP were not yet reported. Our aim was to define the clinicopathological features of IP with positive anti-EJ autoantibody.Methods: We retrospectively examined 13 IP patients with anti-EJ autoantibody. Twelve patients underwent surgical lung biopsy. Cellular nonspecific interstitial pneumonia (NSIP) pattern (67%) and fibrotic NSIP pattern (33%) were diagnosed. The clinical, radiologic, and pathological data were abstracted from the medical records.Results: Median age was 63 years, with 62% women and 58% never smokers. The common symptom was cough (92%) and dyspnea (54%), and no patients met specific connective tissue disease criteria at the first visit. Two patients developed dermatomyositis and four did polymyositis later. High-resolution CT showed lowerlung-predominant pleural irregularities, consolidation, peribronchovascular thickening, and traction bronchiectasis with volume loss. The lung biopsies demonstrated plasmacytic infiltration, interstitial cellular infiltration, and organizing pneumonia in all. IP improved in 10 patients with initial steroid therapy, while five of them relapsed.Conclusions: IP patients with anti-EJ autoantibody had lower lung-predominant shadows with volume loss, cellular or fibrotic NSIP patterns, and good response for initial steroid treatment, although relapse was frequent.