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antisynthetase syndrome

Tatiana Cobo-Ibáñez, Francisco-Javier López-Longo, Beatriz Joven, Patricia E Carreira, Santiago Muñoz-Fernández, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente Cubas, Eva Tomero Muriel, Carmen Barbadillo Mateos, Paloma García de la Peña Lefebvre, Alejandro Gomez-Gomez, Laura Barrio Nogal, Ana Pérez, Raquel Almodovar, Leticia Lojo, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, María Jesús García de Yébenes, Laura Nuño-Nuño
OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected...
November 3, 2018: Clinical Rheumatology
Nozomu Matsuda, Shuzo Sato, Ayaka Nemoto, Hitoshi Kubo, Shunsuke Kobayashi, Yoshikazu Ugawa
A 33-year-old woman developed progressive weakness in the proximal limbs with myalgia and morning stiffness. Physical examination revealed low-grade fever, heliotrope eyelids and mechanic's hand. On neurological examination, she showed Medical Research Council grade 4 weakness in the shoulder girdle, proximal limb muscles, and grade 4 weakness in the abdominis muscle according to Daniels's scale. Laboratory tests revealed elevated serum creatine kinase (6,824 IU/l) and positive anti-PL-7 antibody. A needle electromyography study detected short motor unit potentials of myogenic pattern with abundant fibrillations and positive sharp waves...
October 27, 2018: Rinshō Shinkeigaku, Clinical Neurology
Emer R McGrath, Christopher T Doughty, Anthony A Amato
The major forms of autoimmune myopathies include dermatomyositis (DM), polymyositis (PM), myositis associated with antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). While each of these conditions has unique clinical and histopathological features, they all share an immune-mediated component. These conditions can occur in isolation or can be associated with systemic malignancies or connective tissue disorders (overlap syndromes). As more has been learned about these conditions, it has become clear that traditional classification schemes do not adequately group patients according to shared clinical features and prognosis...
October 16, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Paula Fernández-Rodríguez, Juan José Martín-Marcuartu, Jose Manuel Jiménez-Hoyuela
We present a 44-year-old woman with scapular and pelvic muscle weakness, joint inflammation, and fever. Bone scintigraphy showed high uptake in proximal regions of upper and lower limbs, suspecting inflammatory myopathy and polyarticular damage. These features were the clue to request other complementary tests such as anti-aminoacyl-tRNA-synthetase antibodies and chest CT, which showed interstitial lung disease, defining an antisynthetase syndrome. Therefore, BS allows an earlier diagnosis of inflammatory muscle disease and to identify the optimal site for muscle biopsy...
October 15, 2018: Clinical Nuclear Medicine
Thouraya Ben Salem, Cherifa Abdelkafi, Mounir Lamloum, Imed Ben Ghorbel, Mohamed Habib Houman
INTRODUCTION: Pulmonary manifestations are frequent in patients with antisynthetase syndrome which is a particular form of inflammatory myopathies. AIM: The aim of this study is to describe clinical features and long term outcome of interstitial lung disease in these patients. METHODS: This is a retrospective descriptive study in an internal medicine department. Patients with antisynthetase syndrome hospitalized from 2000 to 2014 were collected...
February 2018: La Tunisie Médicale
Ameen Jubber, Mudita Tripathi, James Taylor
We report the case of an 80-year-old Caucasian man with PL-12 antibody positive antisynthetase syndrome. He presented with progressive dyspnoea and weight loss, later developing dysphagia, mild proximal muscle weakness and mild sicca symptoms. Investigations revealed interstitial lung disease, inflammatory myopathy and an immunology profile consistent with PL-12 antisynthetase syndrome. Prednisolone and cyclophosphamide resulted in a significant improvement of all his symptoms.
October 14, 2018: BMJ Case Reports
Akinori Uruha, Yves Allenbach, Jean-Luc Charuel, Lucile Musset, Audrey Aussy, Olivier Boyer, Kuberaka Mariampillai, Océane Landon-Cardinal, Camille Rasmussen, Loïs Bolko, Thierry Maisonobe, Sarah Leonard-Louis, Shigeaki Suzuki, Ichizo Nishino, Werner Stenzel, Olivier Benveniste
AIMS: To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analyzing different DM subforms, and to test the superiority of MxA to other markers. METHODS: Immunohistochemistry for MxA and retinoic acid-inducible gene I (RIG-I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti-Mi-2 (n=6), -TIF1-γ (n=10), -NXP2 (n=13), -MDA5 (n=10), or -SAE (n=1) autoantibodies and with no detectable autoantibody (n=17)...
September 28, 2018: Neuropathology and Applied Neurobiology
Laure Gallay, Catherine Gayed, Baptiste Hervier
PURPOSE OF REVIEW: Antisynthetase syndrome (ASyS) is an acquired myopathy characterized by the presence of myositis-specific autoantibodies directed against tRNA-synthetases. ASyS is potentially life threatening due to lung involvement and treatment remains a challenge to date. With symptoms not limited to muscles but also involving lung, skin and joints, ASyS appears specific and has a particular pathogenesis, different from the other inflammatory myopathies. This review is intended to discuss the current understanding of ASyS pathogenesis, pointing its current knowledge and also the crucial prospects that may lead to critical improvement of ASyS care...
November 2018: Current Opinion in Rheumatology
Miroslawa Maria Gorecka, Olga Tummon, Yvonne Smyth, Anthony O'Regan
A 57-year-old special Olympics athlete presented with subacute onset dyspnoea. Baseline investigations revealed troponin T rise and an abnormal ECG, which prompted coronary angiography. This was unremarkable, as was his transthoracic echocardiography (TTE). He re-presented 7 months later with progressive dyspnoea associated with significant weight loss, peripheral oedema and intermittent fevers. Examination revealed bilateral fine end-inspiratory crackles, peripheral oedema and fever. Investigations revealed elevated troponin T and raised inflammatory markers...
August 29, 2018: BMJ Case Reports
Albert Selva-O'Callaghan, Iago Pinal-Fernandez, Ernesto Trallero-Araguás, José César Milisenda, Josep Maria Grau-Junyent, Andrew L Mammen
Inflammatory myopathies, collectively known as myositis, are heterogeneous disorders characterised by muscle inflammation, and frequently accompanied by extramuscular manifestations that affect the skin, lung, and joints. Patients with inflammatory myopathies were previously classified as having dermatomyositis if characteristic rashes accompanied the muscle involvement, and as having polymyositis if no rashes were present. Five main types of inflammatory myopathies are now widely recognised: dermatomyositis, immune-mediated necrotising myopathy, sporadic inclusion-body myositis, overlap myositis (including antisynthetase syndrome), and polymyositis...
September 2018: Lancet Neurology
Yasser Emad, Yasser Ragab, Magdy Abd-Elsalam, Johannes J Rasker
A 52-year-old male patient developed RA in March 2009 at the age of 43, with symmetric polyarthritis and active synovitis affecting hands, knees, ankles and both feet without symptoms or signs suggestive of extra-articular features. Laboratory investigations showed negative RF and positive anti-CCP antibodies, negative ANA, negative anti-dsDNA antibodies; the X-rays of both hands showed typical erosive changes in RA and fulfilled the new ACR/EULAR (2010) criteria of RA. The patient achieved remission on a combination of DMARDs...
August 14, 2018: Reumatología Clinica
Veerle Ide, Xavier Bossuyt, Daniël Blockmans, Ellen De Langhe
Objective: As rheumatoid factor (RF) and anticitrullinated protein antibodies (ACPAs) are not routinely tested in idiopathic inflammatory myositis (IIM), little is known about their prevalence and clinical implications in this patient group. In antisynthetase syndrome (ASS), presence of ACPA is reportedly associated with more severe and erosive arthritis. We aim to retrospectively determine the prevalence of RF and ACPA in a cross-sectional cohort of 121 patients diagnosed with IIM and to assess clinical associations...
2018: RMD Open
Q Maloir, K Ghysen, C von Frenckell, R Louis, J Guiot
Antisynthetase syndrome is a clinical entity characterized by specific anti-aminoacyl-tRNA-synthetase antibodies usually associated with inflammatory myopathy and interstitial lung disease. The classic presentation of the pathology is the pulmonary interstitium involvment, wich commonly determines the global prognosis. The subsequent diagnosis of antisynthetase syndrome in patients with acute respiratory distress syndrome (ARDS) is unusual, even more so when a veino-veinous (VV) extracorporeal membrane oxygenation (ECMO) is required...
July 2018: Revue Médicale de Liège
Yair Levi, Lilach Israeli-Shani, Michael Kuchuk, Gali Epstein Shochet, Matthew Koslow, David Shitrit
OBJECTIVE: Interstitial lung diseases (ILD) form a diverse group of parenchymal lung disorders. Currently, a multidisciplinary team (MDT) including pulmonologists, radiologists, and pathologists is the gold standard for ILD diagnosis. Recently, additional subtypes of connective tissue disease (CTD)-ILD with autoimmune features were defined, making the rheumatological assessment increasingly important. We aimed to assess the effect of adding a rheumatologist to the MDT for routine rheumatology assessment...
November 2018: Journal of Rheumatology
Priscille Couture, Pierre-Yves Brillet, Stéphane Varin, Benoît Le Goff, Alain Meyer, Jean Sibilia, Stéphane Jouneau, Dominique Valeyre, Baptiste Hervier, Yurdagül Uzunhan
OBJECTIVE: To investigate the uncommon co-occurrence of antisynthetase syndrome (AS) and sarcoidosis. METHODS: From 2000 to 2015, patients with sarcoidosis were extracted from a retrospective multicentric cohort of 352 patients with AS. RESULTS: Ten patients (2.8%; 6 men, 8 whites, 5 smokers, median age 50 yrs) had both AS and sarcoidosis. Most of the time, sarcoidosis and AS occurred simultaneously (n = 7). Antibody testing revealed anti-Jo1 (n = 5), anti-PL12 (n = 4), or anti-PL7 (n = 1)...
August 2018: Journal of Rheumatology
Carrie Richardson, Uzma Jalal Haque
No abstract text is available yet for this article.
May 4, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Rebecca De Lorenzo, Iago Pinal-Fernandez, Wilson Huang, Jemima Albayda, Eleni Tiniakou, Cheilonda Johnson, Jose C Milisenda, Maria Casal-Dominguez, Andrea M Corse, Sonye K Danoff, Lisa Christopher-Stine, Julie J Paik, Andrew L Mammen
OBJECTIVE: To define the clinical features of myositis patients with anti-PM/Scl-75 and/or anti-PM/Scl-100 autoantibodies at disease onset and during the course of disease and compare them to patients with other forms of myositis. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up were compared between anti-PM/Scl-positive patients and those with the antisynthetase syndrome (AS), dermatomyositis (DM), and immune-mediated necrotizing myopathy (IMNM)...
June 5, 2018: Neurology
Paula A O Araujo, Marilda Guimarães Silva, Eduardo Ferreira Borba, Samuel K Shinjo
OBJECTIVES: A high frequency of metabolic syndrome (MetS) has been recently described in different idiopathic inflammatory myopathies, but not in antisynthetase syndrome (ASS). Therefore, the aim of the present study was to determine the prevalence of MetS in ASS and also its possible association with cardiovascular the risk factors and ASS-related disease characteristics. METHODS: A cross-sectional single centre study of 42 consecutive ASS patients was conducted from 2012 to 2015 and compared to 84 healthy individuals matched for gender, age, ethnicity and body mass index-matched (control group)...
March 2018: Clinical and Experimental Rheumatology
Adam Amlani, Amy Bromley, Aurore Fifi-Mah
A 69-year-old female with antisynthetase syndrome, a history of multiple recurrent infections, and documented previous negative titres for anti-neutrophil cystoplasmic antibody (ANCA) suddenly developed a de novo MPO-ANCA-associated glomerulonephritis three weeks after a fecal microbiota transplantation (FMT) for recurrent Clostridium difficile infections. Six months following her FMT and less than two weeks following treatment for urosepsis, she developed severe cholestasis, a markedly elevated ferritin and hypertriglyceridemia...
2018: Case Reports in Rheumatology
Marwa Mohammed Sabha, Hermann Talom Simo, Rana Mohammed Shadid, Nezam Ibrahim Altorok
Rhabdomyolysis is a syndrome of muscle necrosis with subsequent release of intracellular content into the blood. There are various causes for rhabdomyolysis that include trauma, medications and rarely autoimmune conditions such as autoimmune myositis. Antisynthetase syndrome is an autoimmune condition characterized by positive antisynthetase antibody, myopathy, lung disease and arthritis. To our knowledge, rhabdomyolysis in antisynthetase syndrome has not been reported in the literature. In this report, we present a patient who presented with features of rhabdomyolysis and was diagnosed with antisynthetase syndrome...
June 2018: Rheumatology International
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