Long-term clinical prognosis of anti-aminoacyl-tRNA synthetase antibodies and interstitial lung disease.

INTRODUCTION

Anti-aminoacyl tRNA synthetase (anti-ARS) antibody is the most common myositis-specific antibody subtype. Anti-ARS antibody-positive myositis is often complicated by interstitial lung disease (ILD), but the clinical progression of anti-ARS antibody-positive ILD (ARS-ILD) remains unclear.

METHOD

A prospectively collected, single center longitudinal myositis database was used to retrospectively investigate 131 patients with ARS-ILD based on subtypes of anti-ARS antibodies (Jo-1, PL-7, PL-12, EJ, OJ, and KS). We investigated the occurrence and associated risk factors for pulmonary events, including lung transplantation and pulmonary death, as well as overall mortality at both 5 and 10 years.

RESULTS

This cohort included those with myositis (n = 97), anti-synthetase syndrome without myositis (n = 17), and other connective tissue diseases (n = 17). In a 5-year period, the overall mortality rate and incidence of pulmonary events were both 15%. Across a 10-year timespan, the overall mortality rate increased to 28%, with pulmonary events observed in 24% of cases. A multivariate analysis during the 5-year follow-up, identified poor prognostic factors for overall mortality included dysphagia, dry eyes, usual interstitial pneumonia (UIP) pattern, and the presence of anti-PL-7 antibody. In the 10-year follow-up, dysphagia, diffusing capacity for carbon monoxide (DLCO)%, and anti-PL-7 antibody were associated with increased mortality. Risk factors for pulmonary events at 5 years were DLCO% and UIP pattern, while at 10 years, dysphagia and DLCO% were significant poor prognosis factors.

CONCLUSIONS

Anti-PL-7 antibodies, dysphagia, UIP pattern, and decreased DLCO% predicted poor outcomes in ARS-ILD, indicating the importance of comprehensive risk assessment. Key Points • In patients with ARS-ILD, anti-PL-7 antibodies are associated with risk of all-cause mortality, and evaluation of antibody subtypes in prognosis is important. • The UIP pattern affected prognosis and pulmonary events within the first 5 years. • Dysphagia is the strongest predictor of all-cause mortality and pulmonary events, and management strategies in patients with ARS-ILD are important.