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Rheumatic Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
Patients with DM/PM may have a constellation of clinical findings termed the antisynthetase syndrome. These findings include acute disease onset, constitutional symptoms, myositis, Raynaud's phenomenon, mechanic's hands, non-erosive arthritis and interstitial lung disease.
Inflammatory Myopathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Overlap myositis is a controversial spectrum of IMs that share two or more variants of myositis (PM, PM/DM, and IMNM). The most common overlapping condition is anti-synthetase syndrome (AS), a distinct special variant of myositis-associated autoantibodies. It consists of a typical collection of clinical symptoms including myositis, Raynaud's disease (RD), arthritis, mechanic's hands (MH), interstitial lung disease (ILD), and presence of anti-tRNA synthetase autoantibodies. The most common of the eight anti-synthetase antibodies is anti-Jo1 antibody. Anti-PL-7 and anti-PL-12 are other rare variants found in PM or the PM/DM spectrum (Table 20.1).
Clinical evaluation of the patient with suspected ILD
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Clinicians should maintain a high index of suspicion for IIM, as these patients may present with negative ANA and normal to mildly elevated CK and aldolase levels, or they may fail to demonstrate clinical evidence of myositis and are thus vulnerable to being under-recognized as having an IIM (62,63). Additionally, the ILD may precede development of other supportive features of antisynthetase syndrome (64). Of the myositis-associated autoantibodies, anti-Jo-1 is the most commonly encountered autoantibody in patients with DM and polymyositis (PM). Other prominent anti-tRNA synthetase antibodies include PL-7 and PL-12, which are associated with the presence of ILD (64,65) and less commonly associated with myositis compared to those with anti-Jo-1 positivity (46). Additional anti-tRNA synthetase autoantibodies include KS, OJ, EJ, SC, JS, YRS and ZO, although these are considered rare (66–69). Availability for testing may vary by institution.
Clinical features and diagnostic tools in idiopathic inflammatory myopathies
Published in Critical Reviews in Clinical Laboratory Sciences, 2022
Konstantinos I. Tsamis, Constantinos Boutsoras, Evripidis Kaltsonoudis, Eleftherios Pelechas, Ilias P. Nikas, Yannis V. Simos, Paraskevi V. Voulgari, Ioannis Sarmas
Antisynthetase syndrome (ASS) is a rare idiopathic immune-mediated disorder that can affect multiple systems of the body. Its clinical features extend far beyond muscles and include, apart from myositis, pulmonary involvement, Raynaud’s phenomenon, arthritis, and typical cutaneous lesions [146]. ASS is characterized by the presence of specific anti-synthetase antibodies (anti-aminoacyl tRNA-synthetase, anti-ARS) that are directed against various aminoacyl transfer RNA-synthetases, and results in erroneous protein synthesis. To date, eight RNA-synthetase autoantibodies have been identified; anti-Jo-1, which specifically recognizes histidyl-tRNA-synthetase, is the most common [147]. The association of anti-ARS antibodies with IIMs has been well documented in recent years, with anti-Jo-1 detected in 20–30% of myositis patients [148]. Furthermore, while several case reports have emphasized the association of ASS with malignancies, the data indicates that the incidence of malignancies is lower in ASS than in dermatomyositis [149] and comparable to that seen in the general population [150]. The occurrence of myocarditis in ASS has also been reported [151,152] and Dieval et al. has estimated it at 3.4% of ASS cases [153].
Clinical characteristics and outcome in patients with antisynthetase syndrome associated interstitial lung disease: a retrospective cohort study
Published in European Clinical Respiratory Journal, 2019
Mads Lynge Jensen, Anders Løkke, Ole Hilberg, Charlotte Hyldgaard, Elisabeth Bendstrup, Dan Tran
Antisynthetase syndrome (AS) is defined by the occurrence of IgG anti-aminoacyl RNA-synthetase (anti-ARS) antibodies and associated types of autoimmune manifestations, mainly myositis. The autoimmunity accounts for important clinical manifestations, such as interstitial lung disease (ILD), arthritis, fever, Raynaud´s phenomenon and mechanic’s hand, which can be present at disease onset or appear later as the disease progresses [1]. At present, eight anti-aminoacyl tRNA-synthetase antibodies (anti-ARS) have been identified: anti-Jo1 (histidyl), anti-PL7 (threonyl), anti-PL12 (alanyl), anti-EJ (glycyl), anti-OJ (isoleucyl), anti-KS (asparaginyl), anti-ZO (phenylalanyl) and anti-YRS/HA (tyrosyl). The most commonly encountered antibody is anti-Jo1 that accounts for up to 60–80% [2–4]. ARS are cytoplasmic enzymes that play a vital role in protein synthesis. Defect of these enzymes can theoretically cause adverse events from every organ. AS is a rare, but probably underdiagnosed disease with a reported prevalence for Caucasians of 87/100,000 in a Norwegian study [5]. The reported incidence was 6–10 per million inhabitants per year diagnosed by presence of anti-ARS and polymyositis/dermatomyositis (PM/DM). Approximately, 25–30% of PM/DM patients had anti-ARS [5]. In a US population, the incidence of AS is considered as low as one in 3–4 million with a diagnostic delay of more than 2 years [6].
The antisynthetase syndrome
Published in Baylor University Medical Center Proceedings, 2020
Antisynthetase syndrome (ASyS) is an autoimmune disease affecting multiple organ systems that was first described by Marguerie et al as a triad of polymyositis, interstitial lung disease (ILD), and the presence of myositis-specific autoantibodies to aminoacyl-transfer ribonucleic acid synthetases.1–4 The most specific autoantibody is anti-Jo-1, directed against histidyl-transfer ribonucleic acid-synthetase.1 Other antibodies include anti-PL7, anti-PL12, anti-EJ, anti-OJ, anti-KS, anti-Zo, and anti-Tyr.5 Among idiopathic inflammatory myopathies, ASyS has a higher prevalence of ILD.2,5