A Narrative Review of Interstitial Lung Disease in Anti-Synthetase Syndrome: A Clinical Approach

Authors

Naina Sawal
Sanjay Mukhopadhyay
Sheetal Rayancha
Alastair Moore
Puneet Garcha
Anupam Kumar
Viren Kaul

Publication Date

9-1-2021

Journal

Journal of Thoracic Disease

DOI

10.21037/jtd-20-3328

PMID

34659821

PMCID

PMC8482343

PubMedCentral® Posted Date

9-13-2021

PubMedCentral® Full Text Version

Post-print

Published Open-Access

no

Keywords

Antisynthetase syndrome, interstitial lung disease, computed tomography, immunosuppressive agents, autoimmune disease

Abstract

Anti-synthetase syndrome (AS) is a rare autoimmune disorder characterized by the presence of aminoacyl-transfer RNA synthetase antibodies in conjunction with clinical features such as interstitial lung disease (ILD), Raynaud's phenomenon, nonerosive arthritis, and myopathy. AS distinguishes itself from other inflammatory myopathies by its significant lung involvement and rapidly progressive interstitial lung disease (AS-ILD), therefore the management of AS-ILD requires careful clinical, serologic and radiologic assessment. Glucocorticoids are considered the mainstay of therapy; however, additional immunosuppressive agents are often required to achieve disease control. Patient prognosis is highly dependent on early diagnosis and symptom recognition as the antibody profile is thought to influence therapy response. Since progressive ILD is the leading cause of morbidity and mortality, this review will discuss the clinical approach to patient with suspected AS, with particular emphasis on diagnosis and management of AS-ILD.

Comments

Associated Data