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antisynthetase syndrome

Fernando Henrique Carlos de Souza, Renata Miossi, Júlio Cesar Bertacini de Moraes, Eloisa Bonfá, Samuel Katsuyuki Shinjo
BACKGROUND: Interpretation of rituximab efficacy for refractory idiopathic inflammatory myopathies (IIM) is hampered by the absence of a uniform definition of refractory myositis and clinical response. Therefore, rigorous criteria of refractoriness, together with a homogenous definition of clinical improvement, were used to evaluate rituximab one-year response. METHODS: A retrospective cohort study including 43 IIM (15 antisynthetase syndrome, 16 dermatomyositis, 12 polymyositis) was conducted...
September 18, 2018: Advances in rheumatology
Amornrat Rungcharunthanakul, Nattaporn Sampattavanich
Panniculitis is rarely seen in polymyositis. To the best of our knowledge, we report the first case of panniculitis which precedes the antisynthetase syndrome.
January 2019: Clinical Case Reports
Karim Asi, Anand Gourishankar, Ankur Kamdar
BACKGROUND: Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. However, it has yet to be reported in the IIMs or antisynthetase syndrome. We report a unique case of a patient with IIM, antisynthetase syndrome and coronary artery dilation. CASE PRESENTATION: We report an adolescent presenting with joint symptoms, fever, and eye swelling with a clinical diagnosis of Juvenile Dermatomyositis...
January 10, 2019: Pediatric Rheumatology Online Journal
Kumiko Hayashi, Yoko Machida, Yuki Katayama, Hiroaki Yokote, Kazuyuki Saito, Mayumi Masumura, Akiko Miyashita, Masaki Kobayashi, Shuta Toru
A 69-year-old man was admitted with neck muscle weakness, symmetric proximal muscle weakness, skin rash and elevated serum creatine kinase levels. Muscle biopsy showed perifascicular necrosis and perimysial alkaline phosphatase activity. Chest CT revealed interstitial lung disease and colorectal cancer was diagnosed on colonoscopy. He was serologically positive for anti-EJ antibody, leading to the diagnosis of antisynthetase syndrome (ASS). After laparoscopic low anterior resection of the rectum, he received intravenous methylprednisolone (1,000 mg/d for 3 days) followed by oral prednisolone (50 mg/d)...
December 29, 2018: Rinshō Shinkeigaku, Clinical Neurology
L Gusdorf, C Morruzzi, J Goetz, D Lipsker, J Sibilia, B Cribier
BACKGROUND: Antisynthetase antibodies (ASA) are directed against aminoacyl-tRNA-synthetases, ubiquitous enzymes of which eight types have hitherto been described. They are seen primarily in antisynthetase syndrome (ASS), in which diffuse interstitial lung disease is associated with inflammatory myopathy, joint involvement and cutaneous signs, in particular mechanic's hands. The aim of this study was to determine the prevalence and semiological characteristics of cutaneous involvement in patients presenting ASA...
December 28, 2018: Annales de Dermatologie et de Vénéréologie
G Cassone, M Sebastiani, L Cavagna, K Triantafyllias, V Codullo, C Salvarani, A Manfredi
A 57-year-old woman with a diagnosis of antisynthetase syndrome (ASSD) underwent a nailfold videocapillaroscopy (NVC) showing a scleroderma pattern. Alterations in capillary morphology have been reported in adults with inflammatory myositis (IM) but only recently have the differences in NVC findings between these two diseases been established. ASSD is currently classified as a subset of IM, for which reason only a few studies in literature evaluate its specific hallmarks, showing nonspecific features of NVC in patients with polymyositis and dermatomyositis (DM) and antisynthetase antibodies...
December 20, 2018: Reumatismo
Albrecht Betrains, Ellen De Langhe, Laurens De Saedeleer, Steven Vanderschueren
The antisynthetase syndrome (AS) is a rare auto-immune disease characterised by inflammatory myopathies, interstitial lung disease, inflammatory arthritis, Mechanic's Hands and Raynaud phenomenon. AS infrequently presents with life-threatening lung disease as its primary or sole manifestation. By means of two clinical case reports, an overview is given of recent advances in diagnosis and treatment of AS-related interstitial lung disease. We emphasise the importance of considering AS as a differential diagnosis if lung disease does not respond to standard treatment in a relatively young patient, certainly if there is even a mild suggestion of myopathy, arthritis or cutaneous involvement...
November 27, 2018: Nederlands Tijdschrift Voor Geneeskunde
Katalin Szabó, Levente Bodoki, Melinda Nagy-Vincze, Anett Vincze, Erika Zilahi, Peter Szodoray, Katalin Dankó, Zoltán Griger
The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud's phenomenon, 43% fever, 33% mechanic's hand, and 12% dysphagia...
2018: BioMed Research International
Marco Sebastiani, Konstantinos Triantafyllias, Andreina Manfredi, Miguel Angel González-Gay, Natalia Palmou-Fontana, Giulia Cassone, Ulrich Drott, Christiane Delbrück, Jorge Rojas-Serrano, Chiara Bertolazzi, Laura Nuño, Margherita Giannini, Florenzo Iannone, Esther F Vicente, Santos Castañeda, Albert Selva-O'Callaghan, Ernesto Trallero Araguas, Giacomo Emmi, Annamaria Iuliano, Jutta Bauhammer, Nikolaus Miehle, Simone Parisi, Lorenzo Cavagna, Veronica Codullo, Carlomaurizio Montecucco, Francisco Javier Lopez-Longo, Julia Martínez-Barrio, Juan Carlos Nieto-González, Silvia Vichi, Marco Confalonieri, Paola Tomietto, Raoul Bergner, Alberto Sulli, Francesco Bonella, Federica Furini, Carlo Alberto Scirè, Alessandra Bortoluzzi, Christof Specker, Simone Barsotti, Rossella Neri, Marta Mosca, Marzia Caproni, Julia Weinmann-Menke, Andreas Schwarting, Vanessa Smith, Maurizio Cutolo
OBJECTIVE: To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. METHODS: We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern...
November 15, 2018: Journal of Rheumatology
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 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...
December 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
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