TY - JOUR
T1 - A narrative review of interstitial lung disease in anti-synthetase syndrome
T2 - A clinical approach
AU - Sawal, Naina
AU - Mukhopadhyay, Sanjay
AU - Rayancha, Sheetal
AU - Moore, Alastair
AU - Garcha, Puneet
AU - Kumar, Anupam
AU - Kaul, Viren
N1 - Publisher Copyright:
© 2021 AME Publishing Company. All rights reserved.
PY - 2021/9
Y1 - 2021/9
N2 - 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.
AB - 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.
KW - Antisynthetase syndrome
KW - Autoimmune disease
KW - Computed tomography
KW - Immunosuppressive agents
KW - Interstitial lung disease
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U2 - 10.21037/jtd-20-3328
DO - 10.21037/jtd-20-3328
M3 - Review article
AN - SCOPUS:85116027431
SN - 2072-1439
VL - 13
SP - 5556
EP - 5571
JO - Journal of Thoracic Disease
JF - Journal of Thoracic Disease
IS - 9
ER -