A narrative review of interstitial lung disease in anti-synthetase syndrome: A clinical approach

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

Research output: Contribution to journalReview articlepeer-review

12 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)5556-5571
Number of pages16
JournalJournal of Thoracic Disease
Volume13
Issue number9
DOIs
StatePublished - Sep 2021
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2021 AME Publishing Company. All rights reserved.

Keywords

  • Antisynthetase syndrome
  • Autoimmune disease
  • Computed tomography
  • Immunosuppressive agents
  • Interstitial lung disease

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