Clinical, radiological, and pathological features of anti-asparaginyl tRNA synthetase antibody-related interstitial lung disease

Naoto Aiko, Hideaki Yamakawa, Tae Iwasawa, Tamiko Takemura, Koji Okudela, Hideya Kitamura, Eri Hagiwara, Satoshi Ikeda, Tomohisa Baba, Shinichiro Iso, Yukie Yamaguchi, Yasushi Kondo, Takayoshi Kurabayashi, Kenichi Ohashi, Shinji Sato, Takashi Ogura

Research output: Contribution to journalArticlepeer-review


Background: Myositis and interstitial lung disease (ILD) frequently occur in patients with anti-aminoacyl-tRNA synthetase (ARS) antibodies. Nearly half of ARS-ILD patients have the acute or subacute form of the disease, and one-third of these patients show a deterioration in pulmonary function over the long-term course because of frequent recurrences and refractoriness to therapy. Several reports recently described different characteristics depending on the individual anti-ARS antibodies, and the anti-asparaginyl tRNA synthetase (KS) antibody was strongly linked to ILD rather than to myositis. We therefore hypothesized that KS-ILD may have clinical characteristics that differ from those of other ARS-ILDs. The aim of this study was to clarify the clinical, radiological, and pathological features of KS antibody-positive ILD. Methods: We retrospectively analyzed 19 consecutive patients with KS-ILD who underwent initial clinical measurements and high-resolution computed tomography and pathological assessments. We also analyzed disease behavior based on pulmonary function test results during the follow-up period. Results: Our KS-ILD cohort included patients with dermatomyositis (10.5%), primary Sjögren syndrome (5.3%), and idiopathic ILD (84.2%). Most patients presented with chronic onset (89.5%) and a nonspecific pattern of interstitial pneumonia at each radiological and pathological assessment (89.4% and 85.7%, respectively). The pulmonary function test results showed that the mean changes from the initial %forced vital capacity and %diffusing capacity of the lung for carbon monoxide at 3 years were 3.7% ± 2.9% and 9.35% ± 3.0%, respectively. Conclusions: Most KS-ILD patients showed a tendency for chronic disease onset and long-term stabilization of pulmonary function.

Original languageEnglish
Pages (from-to)196-203
Number of pages8
JournalRespiratory Investigation
Issue number3
Publication statusPublished - 2020 May
Externally publishedYes


  • Anti-asparaginyl tRNA synthetase (anti-KS) antibody
  • Anti-synthetase syndrome
  • Dermatomyositis
  • Idiopathic interstitial pneumonia

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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