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antisynthetase syndrome

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https://www.readbyqxmd.com/read/28855174/the-euromyositis-registry-an-international-collaborative-tool-to-facilitate-myositis-research
#1
James B Lilleker, Jiri Vencovsky, Guochun Wang, Lucy R Wedderburn, Louise Pyndt Diederichsen, Jens Schmidt, Paula Oakley, Olivier Benveniste, Maria Giovanna Danieli, Katalin Danko, Nguyen Thi Phuong Thuy, Monica Vazquez-Del Mercado, Helena Andersson, Boel De Paepe, Jan L deBleecker, Britta Maurer, Liza J McCann, Nicolo Pipitone, Neil McHugh, Zoe E Betteridge, Paul New, Robert G Cooper, William E Ollier, Janine A Lamb, Niels Steen Krogh, Ingrid E Lundberg, Hector Chinoy
AIMS: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data. METHODS: Cross-sectional analysis of IIM cases from 11 countries was performed. Associations between clinical subtypes, extramuscular involvement, environmental exposures and medications were investigated. RESULTS: Of 3067 IIM cases, 69% were female. The most common IIM subtype was dermatomyositis (DM) (31%)...
August 30, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28816394/a-review-of-inflammatory-idiopathic-myopathy-focusing-on-polymyositis
#2
REVIEW
K E N Clark, D A Isenberg
Inflammatory idiopathic myopathies are a group of autoimmune diseases affecting predominantly the proximal skeletal muscles, with raised muscle enzymes, with or without skin involvement and extramuscular organ involvement. Autoantibodies help to characterize patients into different clinical phenotypes. Successful treatment necessitates controlling inflammation early with corticosteroids and invariably requires additional immunosuppressive therapy. This review focuses on the aetiology, pathogenesis, clinical presentation, investigations and management of patients presenting with inflammatory idiopathic myopathies, predominantly focusing on polymyositis and antisynthetase syndrome...
August 17, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28796005/clinical-spectrum-of-anti-jo-1-associated-disease
#3
Sara Monti, Carlomaurizio Montecucco, Lorenzo Cavagna
PURPOSE OF REVIEW: To provide the most recent evidence on anti-Jo-1 syndrome. RECENT FINDINGS: Several new evidences on anti-Jo-1 syndrome have recently emerged. It has been clearly established that, at disease onset, the classic clinical triad (arthritis, myositis and interstitial lung disease - ILD) is only rarely observed. Indeed, disease onset with an isolated arthritis is common. Patients presenting with an isolated manifestation are at high risk for the subsequent occurrence of initially lacking triad findings...
August 8, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28770709/timing-of-onset-affects-arthritis-presentation-pattern-in-antisyntethase-syndrome
#4
Miguel A González-Gay, Carlomaurizio Montecucco, Albert Selva-O'Callaghan, Ernesto Trallero-Araguas, Ovynd Molberg, Helena Andersson, Jorge Rojas-Serrano, Diana Isabel Perez-Roman, Jutta Bauhammer, Christoph Fiehn, Rossella Neri, Simone Barsotti, Hannes M Lorenz, Andrea Doria, Anna Ghirardello, Florenzo Iannone, Margherita Giannini, Franco Franceschini, Ilaria Cavazzana, Konstantinos Triantafyllias, Maurizio Benucci, Maria Infantino, Mariangela Manfredi, Fabrizio Conti, Andreas Schwarting, Giandomenico Sebastiani, Annamaria Iuliano, Giacomo Emmi, Elena Silvestri, Marcello Govoni, Carlo Alberto Scirè, Federica Furini, Francisco Javier Lopez-Longo, Julia Martínez-Barrio, Marco Sebastiani, Andreina Manfredi, Javier Bachiller-Corral, Walter Alberto Sifuentes Giraldo, Marco A Cimmino, Claudio Cosso, Alessandro Belotti Masserini, Giovanni Cagnotto, Veronica Codullo, Mariaeva Romano, Giuseppe Paolazzi, Raffaele Pellerito, Lesley Ann Saketkoo, Norberto Ortego-Centeno, Luca Quartuccio, Alberto Batticciotto, Elena Bartoloni Bocci, Roberto Gerli, Christof Specker, Elena Bravi, Carlo Selmi, Simone Parisi, Fausto Salaffi, Federica Meloni, Enrico Marchioni, Alberto Pesci, Giulia Dei, Marco Confalonieri, Paola Tomietto, Laura Nuno, Francesco Bonella, Nicolò Pipitone, Antonio Mera-Valera, Nair Perez-Gomez, Simone Gerzeli, Raquel Lopez-Mejias, Carlo Jorge Matos-Costa, Jose Antonio Pereira da Silva, José Cifrian, Claudia Alpini, Ignazio Olivieri, María Ángeles Blázquez Cañamero, Ana Belén Rodriguez Cambrón, Santos Castañeda, Lorenzo Cavagna
OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2)...
July 26, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28738907/rig-i-expression-in-perifascicular-myofibers-is-a-reliable-biomarker-of-dermatomyositis
#5
Xavier Suárez-Calvet, Eduard Gallardo, Iago Pinal-Fernandez, Noemi De Luna, Cinta Lleixà, Jordi Díaz-Manera, Ricardo Rojas-García, Ivan Castellví, M Angeles Martínez, Josep M Grau, Albert Selva-O'Callaghan, Isabel Illa
BACKGROUND: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. Perifascicular atrophy in the muscle biopsy is considered a hallmark of DM. However, perifascicular atrophy is not observed in all patients with DM and, conversely, perifascicular atrophy can be observed in other myositis such as antisynthetase syndrome (ASS), complicating DM diagnosis...
July 24, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28638428/acute-respiratory-failure-as-the-first-manifestation-of-antisynthetase-syndrome
#6
Sonia Toujani, Amani Ben Mansour, Meriem Mjid, Abir Hedhli, Jouda Cherif, Yassine Ouahchy, Majed Beji
We report the case of a 40-year-old man with acute respiratory failure syndrome that later proved to be an initial manifestation of antisynthetase syndrome. The diagnosis of this rare combination of a connective tissue disease and an acute respiratory failure is difficult in a previously asymptomatic patient. Early diagnosis and immunosuppressive therapy started precociously prevented the disease progression and resulted in a good outcome.
2017: Tanaffos
https://www.readbyqxmd.com/read/28586844/skeletal-muscle-involvement-in-antisynthetase-syndrome
#7
Eri Noguchi, Akinori Uruha, Shigeaki Suzuki, Kohei Hamanaka, Yuko Ohnuki, Jun Tsugawa, Yurika Watanabe, Jin Nakahara, Takashi Shiina, Norihiro Suzuki, Ichizo Nishino
Importance: Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients' serum. However, antisynthetase syndrome is not included in the histological subsets of idiopathic inflammatory myopathies. Objective: To elucidate the clinical features of myositis in patients with antisynthetase syndrome...
August 1, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28492593/eruptive-melanocytic-nevi-during-azathioprine-therapy-for-antisynthetase-syndrome
#8
Stephanie A Steinweg, Christian R Halvorson, Grace F Kao, Sridhar Dronavalli
Eruptive melanocytic nevi (EMN) are rare multiple benign melanocytic nevi that develop within a few months. The phenomenon has been associated with a variety of dermatologic and systemic conditions, including Stevens-Johnson syndrome, toxic epidermal necrolysis, epidermolysis bullosa, Addison disease, human immunodeficiency virus infection, and internal malignancy, among others. It also is commonly attributed to medications, particularly immunosuppressive and chemotherapeutic agents. We report a case of EMN in a 50-year-old man undergoing azathioprine therapy for antisynthetase syndrome...
April 2017: Cutis; Cutaneous Medicine for the Practitioner
https://www.readbyqxmd.com/read/28479486/integrated-diagnosis-project-for-inflammatory-myopathies-an-association-between-autoantibodies-and-muscle-pathology
#9
REVIEW
Shigeaki Suzuki, Akinori Uruha, Norihiro Suzuki, Ichizo Nishino
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. The clinical phenotypes of inflammatory myopathies are characterized by various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, since the correlation between histological features and autoantibodies had not been fully elucidated, we created the "Integrated Diagnosis Project for Inflammatory Myopathies" in October 2010...
May 4, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28461832/new-insights-into-antisynthetase-syndrome
#10
Manole Cojocaru, Inimioara Mihaela Cojocaru, Bogdan Chicos
Antisynthetase syndrome (ASS) is a rare chronic autoimmune disorder (2-3 times more common in women than in men), associated with interstitial lung disease (the most important feature), dermatomyositis (DM), and polymyositis (PM). The cause of ASS is unknown. Recent developments in immunology have improved our knowledge and it is now possible to classify ASS according to the presence of myositis specific autoantibodies. The hallmark of ASS is the presence of serum autoantibodies directed against aminoacyl-tRNA synthetases (anti-ARS involved in protein synthesis)...
June 2016: Mædica
https://www.readbyqxmd.com/read/28461650/clinical-profiles-and-prognosis-of-patients-with-distinct-antisynthetase-autoantibodies
#11
Jingli Shi, Shanshan Li, Hanbo Yang, Yamei Zhang, Qinglin Peng, Xin Lu, Guochun Wang
OBJECTIVE: To compare the clinical characteristics and identify the longterm outcomes of Chinese patients with different antisynthetase antibodies. METHODS: We investigated retrospectively 124 consecutive patients with antisynthetase syndrome. Medical records, laboratory results, and computed tomography images were obtained. RESULTS: The antisynthetase antibodies we investigated were anti-Jo1 (n = 62), anti-PL7 (n = 31), anti-PL12 (n = 12), and anti-EJ (n = 19)...
July 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28447208/squamous-cell-carcinoma-of-the-lung-associated-with-anti-jo1-antisynthetase-syndrome-a-case-report-and-review-of-the-literature
#12
REVIEW
G Boleto, J-M Perotin, J-P Eschard, J-H Salmon
Antisynthetase syndrome is a heterogeneous idiopathic inflammatory myopathy. Anti-Jo1 is the most common antibody found in this condition. Dermatomyositis is known to be associated with malignancy, but the association between antisynthetase syndrome and malignancy is not clearly established. We report a case of an association of squamous cell carcinoma of the lung and anti-Jo1 antisynthetase syndrome. A 67-year-old man presented with polyarthritis, muscle weakness of the pelvic girdle, "mechanic's hands," and weight loss...
July 2017: Rheumatology International
https://www.readbyqxmd.com/read/28439062/a-rare-case-of-necrotizing-myopathy-and-fibrinous-and-organizing-pneumonia-with-anti-ej-antisynthetase-syndrome-and-ssa-antibodies
#13
Muhammad Kashif, Divya Arya, Masooma Niazi, Misbahuddin Khaja
BACKGROUND Idiopathic inflammatory myopathies are autoimmune disorders that can involve the skin, joints, muscles, and lungs. The most common of these disorders are dermatomyositis, polymyositis, overlap syndrome, and inclusion body myositis. Necrotizing autoimmune myopathy is an idiopathic inflammatory myopathy that is rarely associated with Sjögren's syndrome. The most common lung findings associated with anti-EJ antisynthetase syndrome are nonspecific interstitial pneumonia and usual interstitial pneumonia; this condition is rarely associated with fibrinous and organizing pneumonia...
April 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28389987/-hiker-s-feet-a-novel-cutaneous-finding-in-the-inflammatory-myopathies
#14
REVIEW
Jacob T Cox, David M Gullotti, Christopher A Mecoli, Arash H Lahouti, Jemima Albayda, Julie Paik, Cheilonda Johnson, Sonye K Danoff, Andrew L Mammen, Lisa Christopher-Stine
Mechanic's hands is a well-characterized manifestation of select idiopathic inflammatory myopathy (IIM) syndromes. Less well characterized is the hyperkeratosis of the toes and plantar surface of the feet that can also accompany these disorders. We aim to describe common pedal signs in the context of IIM, and suggest that it may be another key feature in the presentation of these syndromes. A cohort of 2145 myositis patient charts gathered since 2003 were retrospectively reviewed using the key search terms "mechanic's feet" and/or "mechanic's foot...
April 7, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28343013/inflammatory-myopathies-a-new-landscape
#15
Alain Meyer, Béatrice Lannes, Joëlle Goetz, Andoni Echaniz-Laguna, Dan Lipsker, Laurent Arnaud, Thierry Martin, Jacques Eric Gottenberg, Bernard Geny, Jean Sibilia
Greater accuracy in clinical descriptions combined with advances in muscle histology and immunology have established that inflammatory myopathies (IMs), similarly to inflammatory rheumatic diseases, constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset of the myopathy vary widely, and the histological findings distinguish at least five different profiles, which may reflect different pathophysiological processes. Most IMs are connective tissue diseases that can affect multiple organs, among which the most common targets are the skin, joints, and lungs...
March 22, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28293456/acute-digital-ischemia-a-rare-presentation-of-antisynthetase-syndrome
#16
Jin Ei Chan, Sandeep Palakodeti, Matthew J Koster
Antisynthetase syndrome (ASS) is recognized as a subgroup of idiopathic inflammatory myopathies (IIMs). It is associated with autoantibodies directed against aminoacyl-transfer ribonucleic acid (tRNA) synthetase enzymes. We report the first case of anti-PL-7/anti-SSA 52kD ASS presenting as acute digital ischemia, an association not described previously. Occlusive vasculopathy is a rare but serious manifestation that can be seen at presentation in patients with ASS and may herald the onset of severe interstitial lung disease (ILD)...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28257650/subcutaneous-immunoglobulins-for-the-treatment-of-a-patient-with-antisynthetase-syndrome-and-secondary-chronic-immunodeficiency-after-anti-cd20-treatment-a-case-report
#17
Patrick Cherin, Christophe de Jaeger, Jean-Charles Crave, Jean-Christophe Delain, Abir Tadmouri, Zahir Amoura
BACKGROUND: Antisynthetase syndrome is a rare and debilitating multiorgan disease characterized by inflammatory myopathy, interstitial lung disease, cutaneous involvement, and frequent chronic inflammation of the joints. Standard treatments include corticosteroids and immunosuppressants. In some cases, treatment resistance may develop. Administration of immunoglobulins intravenously is recommended in patients with drug-resistant antisynthetase syndrome. CASE PRESENTATION: Here, we describe the case of a 56-year-old woman of Algerian origin...
March 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28210638/an-unusual-suspect-causing-hypoxemic-respiratory-failure
#18
Masooma Aqeel, Bjorn Batdorf, Horatiu Olteanu, Jayshil J Patel
Introduction: Antisynthetase syndrome (ASS) is characterized by the presence of anti-Jo-1 antibodies in conjunction with clinical findings of fever, polymyositis-dermatomyositis, and interstitial lung disease (ILD). Inflammatory myopathies carry a high risk of malignancy, but this association is less well outlined in ASS. We present the case of a patient with ASS who developed non-Hodgkin's lymphoma with acute hypoxemic respiratory failure. Case Presentation: A 44-year-old female with ASS presented with acute hypoxemic respiratory failure...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28158871/mechanic-s-hands-lesions-in-antisynthetase-syndrome
#19
V Mohan, A Maiti, S V Cherian
No abstract text is available yet for this article.
June 1, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28096992/development-of-myasthenia-gravis-8-years-after-interstitial-lung-disease-associated-with-antisynthetase-anti-ej-antibody-syndrome
#20
Takashi Ishiguro, Naho Kagiyama, Eriko Kawate, Kyuto Odashima, Yotaro Takaku, Kazuyoshi Kurashima, Noboru Takayanagi
Patients with antisynthetase-positive interstitial lung disease (ILD) alone sometimes develop myositis during follow-up, but myasthenia gravis (MG) overlapping on antisynthetase syndrome is unusual. A 56-year-old woman with ILD and anti-EJ antibody treated for 8 years developed MG. Physicians should consider myositis and MG when patients develop muscle symptoms during follow-up.
January 2017: Clinical Case Reports
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