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anti tRNA synthetase antibody syndrome

Tomoyuki Naito, Yosuke Tanaka, Mitsunori Hino, Akihiko Gemma
A 72-year-old woman was admitted to our hospital and was diagnosed with interstitial pneumonia (IP) associated with amyopathic dermatomyositis (ADM). The patient experienced three acute IP exacerbations in the 7 years that followed, which were each treated and resolved with steroid pulse therapy. The patient was closely examined for respiratory failure with right heart catheterization (RHC), which demonstrated that she had a mean pulmonary artery pressure (mPAP) of 34 mmHg. The patient was thus diagnosed as having pulmonary hypertension (PH) associated with anti-synthetase syndrome (ASS) and was started on bosentan therapy, which led to improvements in mPAP as well as in subjective symptoms over time...
2017: Respiratory Medicine Case Reports
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
Tomohisa Yamaguchi, Akiko Matsunaga, Masamichi Ikawa, Norimichi Shirafuji, Ichizo Nishino, Tadanori Hamano
A 49-year-old woman presented with progressive muscle weakness of the limbs and dysphagia. Her past and family medical history were unremarkable and she did not take statins or any other medications. Laboratory tests showed that serum levels of creatine kinase were elevated (13,565 IU/l) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies were detected in the serum. Other autoantibodies to the nuclear (ANA), RNP, aminoacyl-tRNA synthetases (ARS), and signal recognition particle (SRP) were negative...
March 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
Joachim Sennekamp, Hans-Peter Seelig
BACKGROUND: A 42-year old male patient with anamnesis of bronchial asthma presented himself with exertional dyspnea. High titer antibodies of cytoplasmic pattern in the indirect immunofluorescence test (IIFT) on HEp-2cells, not attributable to common specificities like anti-tRNA-synthetases, anti-signal recognition particle (SRP) or anti-ribosomal proteins (RPs) prompted the molecular biological approach for determination of antigen specificity. In-depth investigation of the patient's clinical settings revealed a lymphocyte predominant Hodgkin's lymphoma, which could be brought to complete remission by chemotherapy...
August 1, 2016: Clinical Laboratory
Aditya Shah, Samir R Patel
Anti-synthetase syndrome (AS) is a clinical entity which is described classically by the triad of interstitial lung disease (ILD), inflammatory myositis and presence of aminoacyl-tRNA synthetase antibodies (ASA). We describe a rare presentation of this condition with regard to the uncharacteristically acute nature of presentation, acute decompensation in clinical condition, development of acute interstitial pneumonitis requiring rescue extracorporeal membrane oxygenation (ECMO) and accompaniment of significant pericardial effusion on presentation, followed by rapid improvement with initiation of steroids...
September 2016: Journal of Clinical Medicine Research
Ines Maria Grazia Piroddi, Gianluca Ferraioli, Cornelius Barlascini, Corrado Castagneto, Antonello Nicolini
Anti-synthetase syndrome (ASS) is defined as a heterogeneous connective tissue disorder characterized by the association of an interstitial lung disease (ILD) with or without inflammatory myositis with the presence of anti-aminoacyl-tRNA-synthetase antibodies. ILD is one of the major extra-muscular manifestations of polymyositis and dermatomyositis. We report a case of a patient with dyspnea, cough, and intermittent fever as well as ILD associated ASS in the absence of muscular involvement. This patient was admitted to the emergency department with severe respiratory failure requiring non-invasive ventilation...
July 2016: Respiratory Investigation
Yuko Waseda, Takeshi Johkoh, Ryoko Egashira, Hiromitsu Sumikawa, Keigo Saeki, Satoshi Watanabe, Ryo Matsunuma, Hazuki Takato, Yukari Ichikawa, Yasuhito Hamaguchi, Akira Shiraki, Yoshinao Muro, Masahide Yasui, Helmut Prosch, Christian Herold, Kazuo Kasahara
OBJECTIVES: To describe the pulmonary CT findings in patients with anti-ARS-antibody-positive interstitial lung disease (anti-ARS-ILD) METHODS: The CT findings of 64 patients with anti-ARS-ILD were retrospectively reviewed. The images were retrospectively reviewed independently by 2 chest radiologists, and the final decision on the CT findings was made by a third chest radiologist. RESULTS: There were 16 male and 48 female patients, aged 54.2±13.4 years. Sixteen patients had anti Jo-1, 24 had anti-EJ, 9 had anti-PL-7, 7 had anti-PL-12, 5 had anti-KS, and 3 had anti-OJ antibodies...
August 2016: European Journal of Radiology
Mineto Ota, Mari Kihara, Akito Takamura, Hitoshi Kohsaka
  Antisynthetase syndrome is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies and characteristic clinical features. We report an anti-EJ antibody-positive case presenting an ILD with slight hyperkeratotic skin changes on the fingertips that appeared simultaneously with respiratory symptoms. We suspected those skin changes of a disease manifestation of antisynthetase syndrome, and thus investigated anti-synthetase antibodies. This case implies that broader spectrum of the patients should fall in antisynthetase syndrome even though the present diagnostic criteria call for mechanic's hand as a skin manifestation...
2016: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
Margherita Giannini, Antonella Notarnicola, Maryam Dastmalchi, Ingrid E Lundberg, Giuseppe Lopalco, Florenzo Iannone
Auto-antibodies against aminoacyl-tRNA-synthetases (anti-ARS Abs) represent the hallmark of the anti-synthetase syndrome that is defined as the clinical association of fever, Raynaud's phenomenon, myositis, interstitial lung disease (ILD), arthritis and mechanic's hands. Recently, differences in clinical features depending on specific anti-ARS Abs have been reported. We describe three cases of anti-EJ (anti-glycyl) antibody-positive patients presenting with ILD as a common feature, but with heterogeneous histopathological and radiographic patterns and with different responses to treatment...
September 2016: Clinical Rheumatology
Hanna D Bremer, Erik Lattwein, Stefanie Renneker, Inger Lilliehöök, Johan Rönnelid, Helene Hansson-Hamlin
Circulating antinuclear antibodies (ANA) are commonly present in the systemic autoimmune disease Systemic Lupus Erythematosus (SLE) and in other systemic rheumatic diseases, in humans as well as in dogs. The indirect immunofluorescence (IIF)-ANA test is the standard method for detecting ANA. Further testing for specific ANA with immunoblot techniques or ELISAs is routinely performed in humans to aid in the diagnosis and monitoring of disease. Several specific ANA identified in humans have been identified also in suspected canine SLE but, in contrast to humans, investigation of autoantibodies in canine SLE is mainly restricted to the IIF-ANA test...
December 15, 2015: Veterinary Immunology and Immunopathology
Kengo Akashi, Jun Saegusa, Akio Morinobu
Connective tissue diseases are chronic inflammatory diseases that can affect multiple organs and, thus, have a broad spectrum of clinical presentations. Various autoantibodies are detected in patients with connective tissue diseases, represented by anti-nuclear antibody for systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), Sjögren's syndrome, and mixed connective tissue disease. Assessment of the autoantibody profile is fundamental for the clinical management of patients with connective tissue diseases, providing important data for the diagnosis, clinical characterization, and disease activity evaluation...
May 2015: Rinsho Byori. the Japanese Journal of Clinical Pathology
Minoru Satoh, Shin Tanaka, Angela Ceribelli, S John Calise, Edward K L Chan
Autoantibodies specific for idiopathic inflammatory myopathy (myositis-specific autoantibodies (MSAs)) are clinically useful biomarkers to help the diagnosis of polymyositis/dermatomyositis (PM/DM). Many of these are also associated with a unique clinical subset of PM/DM, making them useful in predicting and monitoring certain clinical manifestations. Classic MSAs known for over 30 years include antibodies to Jo-1 (histidyl transfer RNA (tRNA) synthetase) and other aminoacyl tRNA synthetases (ARS), anti-Mi-2, and anti-signal recognition particle (SRP)...
February 2017: Clinical Reviews in Allergy & Immunology
Shoichi Fukui, Noritaka Sekiya, Yasunobu Takizawa, Hiroshi Morioka, Hirofumi Kato, Akio Aono, Kinuyo Chikamatsu, Satoshi Mitarai, Satomi Kobayashi, Satoshi Kamei, Keigo Setoguchi
Mycobacterium abscessus is a rapidly growing mycobacterium found mainly in patients with respiratory or cutaneous infections, but it rarely causes disseminated infections. Little is known about the clinical characteristics, treatment, and prognosis of disseminated M abscessus infection. A 75-year-old Japanese woman who had been treated for 17 years with a corticosteroid for antisynthetase syndrome with antithreonyl-tRNA synthetase antibody developed swelling of her right elbow. X-ray of her right elbow joint showed osteolysis, and magnetic resonance imaging revealed fluid in her right elbow joint...
May 2015: Medicine (Baltimore)
Sebastian Schöpp, Saban Elitok, Markus Bieringer, Wolfgang Schneider, Friedrich C Luft
Anti-synthetase syndrome is a relatively recently described auto-immune disease characterized by auto-antibodies to enzymes that acetylate transfer RNA (tRNA). Interstitial pulmonary disease and inflammatory myopathy are regular findings. Our patient also exhibited a lupus-like glomerulonephritis. An important clue was the presence of 'mechanics' hands. Nephrologists need to be aware of this syndrome.
April 2013: Clinical Kidney Journal
Ellen De Langhe, Jan Lenaerts, Xavier Bossuyt, Rene Westhovens, Wim A Wuyts
INTRODUCTION: Interstitial lung disease can be idiopathic or occur in the setting of connective tissue diseases. In the latter case it requires a different treatment approach with a better prognosis. Interstitial lung disease can precede the onset of typical connective tissue disease features by many years, and therefore meticulous multidisciplinary follow-up is crucial. This case highlights the diagnostic challenge and the need for intensified attention for subtle clinical features when faced with interstitial lung disease in patients with characteristics of a hitherto undifferentiated connective tissue disease...
2015: Journal of Medical Case Reports
Lívia Regina Theilacker, Fabíola Sampaio Brandão, Fernanda Velloso Goulart, João Luiz Pereira Vaz, Luiz Octávio Dias D'Almeida, Maria Cecília da Fonseca Salgado
Antissintetase Syndrome (ASS) is characterized by myositis, Raunaud's phenomenon, fever, intertitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1...
March 2015: Revista Brasileira de Reumatologia
Masaomi Tomonaga, Noriho Sakamoto, Yuji Ishimatsu, Tomoyuki Kakugawa, Tatsuhiko Harada, Shota Nakashima, Atsuko Hara, Shintaro Hara, Yoshihiro Horai, Atsushi Kawakami, Hiroshi Mukae, Shigeru Kohno
Anti-PL-7 is an anti-tRNA synthetase antibody, and interstitial lung disease (ILD) is the most frequent complication of anti-PL-7-associated antisynthetase syndrome. However, the features of ILD have not been fully elucidated. The present study retrospectively compares 7 and 15 patients who were positive for anti-PL-7 and anti-Jo-1 antibodies, respectively. The features of ILD did not significantly differ between the two groups, but the ratio of lymphocytes in bronchoalveolar lavage fluid was higher in the Jo-1 than in the PL-7 group...
February 2015: Lung
Guillaume Lefèvre, Alain Meyer, David Launay, Irène Machelart, Michel DeBandt, Julien Michaud, Anne Tournadre, Pascal Godmer, Jean-Emmanuel Kahn, Aurélie Behra-Marsac, Marie-Agnès Timsit, Nicolas Schleinitz, Daniel Wendling, Sylvie Melac-Ducamp, Philippe Boyer, Anne Peretz, Thierry Lequerré, Christophe Richez, Lidwine Stervinou-Wemeau, Sandrine Morell-Dubois, Marc Lambert, Sylvain Dubucquoi, Benoit Wallaert, Olivier Benveniste, René-Marc Flipo, Pierre-Yves Hatron, Jean Sibilia, Eric Hachulla, Baptiste Hervier
OBJECTIVE: The aim of this study was to determine the frequency and characteristics of antisynthetase syndrome (ASS) revealed by polyarthritis. METHODS: First we conducted a retrospective single-centre study to assess the frequency of ASS patients who presented with polyarthritis without pulmonary and/or muscle symptoms. Secondly, we conducted a larger, multicentre study in order to describe the clinical characteristics of these patients. Exclusion criteria were the presence of RF, the presence of ACPA and overlap with another CTD...
May 2015: Rheumatology
Robert W Hallowell, Sonye K Danoff
PURPOSE OF REVIEW: To highlight recent advances in understanding the clinical spectrum, pathogenesis, and treatment of interstitial lung disease associated with inflammatory myositis and the antisynthetase syndrome. RECENT FINDINGS: In recent years, serologic tests to identify the less common antisynthetase antibodies and the anti-MDA-5 antibody have become commercially available. As a result, several large, retrospective analyses have illustrated both the pulmonary and non-pulmonary features associated with the antisynthetase syndrome and myositis-related interstitial lung disease...
November 2014: Current Opinion in Rheumatology
Clara Sciorati, Antonio Esposito, Lara Campana, Tamara Canu, Antonella Monno, Anna Palmisano, Francesco De Cobelli, Alessandro Del Maschio, Dana P Ascheman, Angelo A Manfredi, Patrizia Rovere-Querini
Inflammatory myopathies comprise heterogeneous disorders. Their etiopathogenesis is poorly understood, because of the paucity of informative experimental models and of approaches for the noninvasive study of inflamed tissues. Magnetic resonance imaging (MRI) provides information about the state of the skeletal muscle that reflects various facets of inflammation and remodeling. This technique has been scarcely used in experimental models of inflammatory myopathies. We characterized the performance of MRI in a well-established mouse model of myositis and the antisynthetase syndrome, based on the immunization of wild-type mice with the amino-terminal fragment of histidyl-tRNA synthetase (HisRS)...
2014: BioMed Research International
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