Evolution of Pulmonary Function in a Cohort of Patients with Interstitial Lung Disease and Positive for Antisynthetase Antibodies

J Rheumatol. 2020 Mar;47(3):415-423. doi: 10.3899/jrheum.181141. Epub 2019 Jun 15.

Abstract

Objective: To describe the evolution of the pulmonary function in patients with interstitial lung disease (ILD) who are positive for at least 1 of the antisynthetase antibodies (ASAB) after medical treatment, and to compare whether the evolution of pulmonary function is associated with the type of ASAB.

Methods: Patients with ILD and positive for at least 1 of the ASAB (anti-Jo1, anti-PL7, anti-PL12, anti-EJ, or anti-OJ) were included. The clinical evolution, time until death or censoring, and improvement of lung disease were registered.

Results: The study included 118 patients. Most of the patients had a high extent of ground glass opacities in high-resolution computed tomography (HRCT) and low extent of fibrosis. In the final evaluation of pulmonary function (median 749.5 days of followup), 67% of the patients had lung disease improvement. The improvement occurred within the first 6 months after initiating medical treatment; thereafter, pulmonary function remained stable in most of the patients. A decrease of the extent of ground glass opacities was demonstrated in HRCT at followup in those patients with pulmonary improvement. No differences were observed in the percentage of patients who achieved improvement between the ASAB groups, or in survival.

Conclusion: Improvement of pulmonary function was observed in 67% of the patients. Improvement was observed in all ASAB groups and occurred within 6 months after initiating medical treatment.

Keywords: ANTISYNTHETASE SYNDROME; IDIOPATHIC INFLAMMATORY MYOPATHIES; INTERSTITIAL LUNG DISEASE; MYOSITIS.

MeSH terms

  • Adult
  • Aged
  • Amino Acyl-tRNA Synthetases / immunology*
  • Autoantibodies / blood*
  • Disease Progression
  • Female
  • Fibrosis
  • Follow-Up Studies
  • Humans
  • Immunoglobulin G / blood
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung / physiopathology*
  • Lung Diseases, Interstitial / complications*
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / mortality
  • Male
  • Middle Aged
  • Myositis / blood
  • Myositis / complications*
  • Survival Analysis
  • Tomography Scanners, X-Ray Computed
  • Vital Capacity

Substances

  • Autoantibodies
  • Immunoglobulin G
  • Amino Acyl-tRNA Synthetases

Supplementary concepts

  • Antisynthetase syndrome