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

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.

Muscle Biopsy: A Requirement for Precision Medicine in Adult-Onset Myopathy

“…Subtypes of IIM are highly relevant to the effectiveness of treatments, the frequency of concomitant ILD, and the ratio of concurrent malignancy [ 51 , 52 ]. The accurate diagnosis of IIM subtypes guides clinicians on the frequency and extensiveness of cancer surveillance [ 53 , 54 ] and suggests the prognosis of pulmonary complications [ 37 , 51 , 52 , 55 , 56 , 57 , 58 ]. In addition, unlike most primary muscle diseases, IIMs are treatable and should not be misdiagnosed as other hereditary or degenerative myopathies.…”

Antisynthetase syndrome with rare EJ‐1 antibodies with antiphospholipid syndrome

“…Furthermore, early evaluation with autoimmune screening including myositis-specific antibodies can potentially reduce the need for lung biopsy. 3 ASS is considered a distinct clinical entity of IIM. Until recently, there have existed two main classification criteria by Connors et al and Solomon et al, which require the presence of antibodies to aminoacyl transfer RNA synthetase and the specific clinical features.…”

“…Until recently, there have existed two main classification criteria by Connors et al and Solomon et al, which require the presence of antibodies to aminoacyl transfer RNA synthetase and the specific clinical features 12,13 . Myositis antibodies are often divided into Jo‐1 and non‐Jo‐1 antibodies (anti‐EJ‐1, anti PL‐12, anti‐Pl‐7, anti‐OJ, anti‐KS, anti‐YRS/Ha, anti‐SC, anti‐Zo, anti‐JS), with non‐Jo‐1 patients having worse outcomes and reduced survival 3,14 . Antibody levels can fluctuate based on disease severity and not all laboratories test for all antibodies associated with ASS, thereby adding to the challenges of diagnosis 15 .…”

“…Antibody levels can fluctuate based on disease severity and not all laboratories test for all antibodies associated with ASS, thereby adding to the challenges of diagnosis 15 . The most common radiological findings on chest computed tomography are non‐specific interstitial pneumonia (NSIP) and COP, although UIP patterns are not infrequent 3,15 . Histological specimens are often not utilized in diagnosis, but histopathological findings include diffuse alveolar damage, UIP and NSIP.…”

“…1,2 ASS often presents as isolated ILD making the diagnosis challenging, leading to delays in diagnosis and thereby impacting treatment and prognosis. 3 Treatment options include glucocorticoids, steroid-sparing agents such as mycophenolate and azathioprine, and rituximab in refractory cases. 4 The association with pulmonary embolism has been limited to case studies, and there is limited literature detailing the association with antiphospholipid syndrome (APLS).…”