Interstitial lung disease in patients with antisynthetase syndrome: a retrospective case series study

Jpn J Radiol. 2021 Jan;39(1):40-46. doi: 10.1007/s11604-020-01030-3. Epub 2020 Sep 2.

Abstract

Purpose: Antisynthetase syndrome (ASS) is a rare systemic autoimmune condition associated to the presence of anti-aminoacyl-tRNA synthetase antibodies. Interstitial lung disease (ILD) is the most prevalent manifestation of ASS and is a major determinant of morbidity and mortality. The aim of this study was to describe the radiological characteristics of patients with ASS-associated-ILD in our institution.

Materials and methods: Medical records from 2014 to 2020 were retrospectively reviewed and patients with a diagnosis of ASS and evidence of ILD on HRCT were included. HRCT images were reviewed by two thoracic radiologists in consensus. Five HRCT patterns were defined: cellular non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), mixed NSIP/OP pattern, acute interstitial pneumonia (AIP) pattern and fibrotic pattern. Descriptive statistics was calculated for all variables.

Results: Twenty-two patients with ASS who met inclusion criteria were included. The disease presented with the typical triad of ASS in 45% of patients, 55% had ILD only at the onset. Cellular NSIP was present in 27% of patients, OP in 23%, mixed NSIP/OP in 9%, AIP in 18% and a fibrotic pattern in 23%.

Conclusion: HRCT findings in ASS-associated ILD are often non-specific; nevertheless, it is important to consider this diagnosis, especially in patients presenting with acute onset of symptoms.

Keywords: Acute interstitial pneumonia; Antisynthetase syndrome; HRCT.

MeSH terms

  • Female
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Diseases, Interstitial / complications*
  • Lung Diseases, Interstitial / diagnostic imaging*
  • Lung Diseases, Interstitial / pathology
  • Male
  • Middle Aged
  • Myositis / complications*
  • Myositis / diagnostic imaging*
  • Myositis / pathology
  • Retrospective Studies
  • Tomography, X-Ray Computed / methods*

Supplementary concepts

  • Antisynthetase syndrome