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antisynthetase syndrome

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...
August 15, 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
Tracy J Doyle, Namrata Dhillon, Rachna Madan, Fernanda Cabral, Elaine A Fletcher, Diane C Koontz, Rohit Aggarwal, Juan C Osorio, Ivan O Rosas, Chester V Oddis, Paul F Dellaripa
OBJECTIVE: To assess clinical outcomes including imaging findings on computed tomography (CT), pulmonary function testing (PFT), and glucocorticoid (GC) use in patients with the antisynthetase syndrome (AS) and interstitial lung disease (ILD) treated with rituximab (RTX). METHODS: We retrospectively identified all patients at 2 institutions with AS-ILD who were treated with RTX. Baseline demographics, PFT, and chest CT were assessed before and after RTX. Two radiologists independently evaluated CT using a standardized scoring system...
June 2018: Journal of Rheumatology
Y-H Chiu, C-C Lu, F-C Liu, H-C Chen
No abstract text is available yet for this article.
May 1, 2018: QJM: Monthly Journal of the Association of Physicians
Go Saito, Masato Kono, Akari Tsutsumi, Yu Koyanagi, Koichi Miyashita, Takeshi Kobayashi, Hironao Hozumi, Yoshihiro Miki, Yoshifumi Arai, Yoshiro Otsuki, Dai Hashimoto, Tomoyuki Fujisawa, Toru Nakamura, Takafumi Suda, Hidenori Nakamura
A 68-year-old woman was admitted to our hospital with fever and pleural effusion. Her thoracentesis showed eosinophilic pleural effusion (EPE) without any evidence of malignancy, infection, or trauma. Pleural biopsy revealed pleuritis and intercostal myositis. Characteristic skin manifestations, including Gottron's sign, interstitial lung disease, and pericardial effusion, appeared later in the clinical course. She was finally diagnosed with anti-PL-7 antisynthetase syndrome (ASS) based on the presence of anti-PL-7 antibody, and she fulfilled the diagnostic criteria for dermatomyositis...
August 1, 2018: Internal Medicine
Kimi Okada, Shoji Asakura, Tomofumi Yano, Takumi Kishimoto
Primary effusion lymphoma (PEL) is a rare type of extranodal lymphoma, typically of a B-cell origin, which presents as lymphomatous effusion with no nodal enlargement or tumor masses. The development PEL is universally associated with human herpes virus-8 (HHV-8) infection. Cases of HHV-8-negative primary lymphomatous effusion have recently been reported and referred to as HHV-8-unrelated PEL-like lymphoma. Some cases of this disease have been reported in iatrogenic immunocompromised patients. The mechanisms responsible for the inhibitory effects of the discontinuation of immunosuppressants other than methotrexate (MTX) against the disease, which have been demonstrated for MTX-associated lymphoproliferative disorders, have not yet been elucidated...
September 2018: International Journal of Hematology
Anna Tjärnlund, Matteo Bottai, Ingrid E Lundberg
No abstract text is available yet for this article.
June 2018: Arthritis & Rheumatology
Maud A W Hermans, Jelle R Miedema, Rob M Verdijk, Paul L A van Daele
No abstract text is available yet for this article.
April 1, 2018: Rheumatology
Luis Gorospe, Carlos de la Puente-Bujidos, Isabel García-Gómez-Muriel, Ignacio Barbolla-Díaz, María Ángeles Fernández-Méndez
No abstract text is available yet for this article.
January 9, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Eva De Backer, Félix Gremonprez, Guy Brusselle, Pieter Depuydt, Jo Van Dorpe, Carole Van Haverbeke, Pieter C Goeminne, Eric Derom
CASE PRESENTATION:  We present a case of a 55-year-old Caucasian male with manifestations of dermatomyositis complicated with rapidly progressive interstitial lung disease (RP-ILD). Diagnosis of anti-MDA5 positive dermatomyositis was made. DISCUSSION:  Myositis specific antibodies (MSA) can be used for diagnosis and predicting prognosis in patients with polymyositis and dermatomyositis. Anti-MDA5 positive dermatomyositis should be considered in patients presenting with dermatomyositis and a disease course resembling antisynthetase syndrome in the absence of antisynthetase autoantibodies, especially if a remarkably high ferritin is noted...
December 29, 2017: Acta Clinica Belgica
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