Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Observational Study
. 2022 Jan:191:106432.
doi: 10.1016/j.rmed.2021.106432. Epub 2021 May 4.

Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience

Erin M Wilfong  1 , Jennifer J Young-Glazer  2 , Bret K Sohn  2 , Gabriel Schroeder  3 , Narender Annapureddy  2 , Erin A Gillaspie  4 , April Barnado  2 , Leslie J Crofford  2 , Rosemarie Beckford Dudenhofer  5
Affiliations
Observational Study

Anti-tRNA synthetase syndrome interstitial lung disease: A single center experience

Erin M Wilfong et al. Respir Med. 2022 Jan.

Abstract

Background: Recognition of Anti-tRNA synthetase (ARS) related interstitial lung disease (ILD) is key to ensuring patients have prompt access to immunosuppressive therapies. The purpose of this retrospective cohort study was to identify factors that may delay recognition of ARS-ILD.

Methods: Patients seen at Vanderbilt University Medical Center between 9/17/2017-10/31/2018 were included in this observational cohort. Clinical and laboratory features were obtained via chart abstraction. Kruskal-Wallis ANOVA, Mann-Whitney U, and Fisher's exact t tests were utilized to determine statistical significance.

Results: Patients with ARS were found to have ILD in 51.9% of cases, which was comparable to the frequency of ILD in systemic sclerosis (59.5%). The severity of FVC reduction in ARS (53.2%) was comparable to diffuse cutaneous systemic sclerosis (56.8%, p = 0.48) and greater than dermatomyositis (66.9%, p = 0.005) or limited cutaneous systemic sclerosis (71.8%, p = 0.005). Frank honeycombing was seen with ARS antibodies but not other myositis autoantibodies. ARS patients were more likely to first present to a pulmonary provider in a tertiary care setting (53.6%), likely due to fewer extrapulmonary manifestations. Only 33% of ARS-ILD were anti-nuclear antibody, rheumatoid factor, or anti-cyclic citrullinated peptide positive. Patients with ARS-ILD had a two-fold longer median time to diagnosis compared to other myositis-ILD patients (11.0 months, IQR 8.5-43 months vs. 5.0 months, IQR 3.0-9.0 months, p = 0.003).

Conclusions: ARS patients without prominent extra-pulmonary manifestations are at high risk for not being recognized as having a connective tissue disease related ILD and miscategorized as usual interstitial pneumonia/idiopathic pulmonary fibrosis without comprehensive serologies.

Keywords: Anti-tRNA synthetase syndrome; Connective tissue disease related interstitial lung disease; Idiopathic inflammatory myopathies; Systemic sclerosis; Usual interstitial pneumonia.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Time to diagnosis for patients with ARS-ILD (n=21) compared to DM/PM-ILD (n=14). Violin plots show the range with interquartile range. Mann-Whitney U-tests shown.
Figure 2.
Figure 2.
Physiologic features of IIM-ILD compared to SSc-ILD. (A) Patients with ARS have more severe restriction than DM or lcSSc and similar restriction compared to dcSSc. (B) There is no significant difference in diffusion impairment amongst IIM or SSc subsets. Box plots depict median ± IQR, KW=Kruskal-Wallis, Mann-Whitney U-test *p<0.05, **p<0.01.
Figure 3.
Figure 3.
Stratification of physiologic impairment according to American Thoracic Society criteria. The severity of impairment of (A) FVC and (B) DLCO for IIM and SSc subsets are shown.
See this image and copyright information in PMC

References

    1. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975: 292(7): 344–347. - PubMed
    1. Aggarwal R, Cassidy E, Fertig N, Koontz DC, Lucas M, Ascherman DP, Oddis CV. Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients. Ann Rheum Dis 2014: 73(1): 227–232. - PMC - PubMed
    1. Pinal-Fernandez I, Casal-Dominguez M, Huapaya JA, Albayda J, Paik JJ, Johnson C, Silhan L, Christopher-Stine L, Mammen AL, Danoff SK. A longitudinal cohort study of the anti-synthetase syndrome: increased severity of interstitial lung disease in black patients and patients with anti-PL7 and anti-PL12 autoantibodies. Rheumatology (Oxford) 2017: 56(6): 999–1007. - PMC - PubMed
    1. Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendia-Roldan I, Selman M, Travis WD, Walsh S, Wilson KC, American Thoracic Society ERSJRS, Latin American Thoracic S. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018: 198(5): e44–e68. - PubMed
    1. Hoffmann-Vold A-M, Gunnarsson R, Garen T, Midtvedt Ø, Molberg Ø. Performance of the 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis (SSc) in Large, Well-defined Cohorts of SSc and Mixed Connective Tissue Disease. The Journal of Rheumatology 2015: 42(1): 60–63. - PubMed

Publication types