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myositis-associated interstitial lung disease

Julie Obert, Olivia Freynet, Hilario Nunes, Pierre-Yves Brillet, Makoto Miyara, Robin Dhote, Dominique Valeyre, Jean-Marc Naccache
Interstitial lung disease (ILD) is a common form of extramuscular involvement in patients with polymyositis/dermatomyositis and is associated with poor prognosis. This study was designed to describe the long-term outcome of myositis-associated ILD. This retrospective observational study was conducted in 48 consecutive patients. Two groups defined according to outcome were compared to determine prognostic factors: a "severe" group (vital capacity [VC] < 50 % or carbon monoxide transfer factor [TLCO] < 35 % or death or lung transplantation) and a "nonsevere" group (other patients)...
October 8, 2016: Rheumatology International
S Hoa, M Hudson, Y Troyanov, S Proudman, J Walker, W Stevens, M Nikpour, S Assassi, M D Mayes, M Wang, M Baron, M J Fritzler
Autoantibodies directed against the Ku autoantigen are present in systemic sclerosis (SSc) and have been associated with myositis overlap and interstitial lung disease (ILD). However, there is a paucity of data on the clinical correlates of anti-Ku antibodies in the absence of other SSc-specific antibodies. The aim of this study was to assess the clinical correlates of single-specificity anti-Ku in SSc.An international (Canada, Australia, USA, Mexico) cohort of 2140 SSc subjects was formed, demographic and clinical variables were harmonized, and sera were tested for anti-Ku using a line immunoassay...
August 2016: Medicine (Baltimore)
Cody M Lee, Diana Girnita, Arundhati Sharma, Surabhi Khanna, Jean M Elwing
Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia...
2016: Case Reports in Rheumatology
Baptiste Hervier, Mikaël Perez, Yves Allenbach, Hervé Devilliers, Fleur Cohen, Yurdagül Uzunhan, Hanane Ouakrim, Karim Dorgham, Jean-François Méritet, Elisabeth Longchampt, Werner Stenzel, Isabelle Cremer, Olivier Benveniste, Vincent Vieillard
Antisynthetase syndrome (aSS) is characterized by the association of interstitial lung disease and myositis with anti-tRNA synthetase autoantibodies. Immune mechanisms leading to aSS could be initiated in the lungs, but the role of NK cells has not yet been studied. Both extensive NK cell phenotype and functions were compared between 33 patients and 26 controls. Direct and redirected polyfunctionality assays (degranulation and intracellular production of TNF-α and IFN-γ) were performed spontaneously or after IL-12 plus IL-18 stimulation in the presence of K562 or P815 target cells, respectively...
September 1, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
Shin Ohta, Ki-Ichi Unoda, Hideto Nakajima, Soichiro Ikeda, Yasuhito Hamaguchi, Fumiharu Kimura
Myositis-specific autoantibodies (MSAs) are associated with myositis. Anti-nuclear matrix protein 2 (NXP-2) antibody was recently identified as a major MSA and was observed mostly in juvenile dermatomyositis. We report the case of a 44-year-old man who presented with myopathy with anti-NXP-2 antibody and large cell carcinoma of the lung. He was hospitalized because of myalgia and edema of limbs. Neurological examination revealed mild proximal-dominant weakness in all four extremities, and laboratory studies showed elevated creatine kinase level (6,432 IU/l)...
August 31, 2016: Rinshō Shinkeigaku, Clinical Neurology
Seo-Hyun Kim, I-Nae Park
Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, mechanic's hand-like cutaneous involvement, Raynaud phenomenon, and polyarthritis. Lung disease is the presenting feature in 50% of the cases. We report a case of a 60-year-old female with acute respiratory distress syndrome (ARDS), which later proved to be an unexpected and initial manifestation of anti-Jo-1 antibody-positive antisynthetase syndrome...
July 2016: Tuberculosis and Respiratory Diseases
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
Ragnar Gunnarsson, Siri Opsahl Hetlevik, Vibke Lilleby, Øyvind Molberg
The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3...
February 2016: Best Practice & Research. Clinical Rheumatology
R Nakashima, Y Hosono, T Mimori
Anti-aminoacyl-tRNA synthetase (ARS) and anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are closely associated with interstitial lung disease in polymyositis and dermatomyositis. Anti-ARS-positive patients develop common clinical characteristics termed anti-synthetase syndrome and share a common clinical course, in which they respond well to initial treatment with glucocorticoids but in which disease tends to recur when glucocorticoids are tapered. Anti-MDA5 antibody is associated with rapidly progressive interstitial lung disease and poor prognosis, particularly in Asia...
July 2016: Lupus
Shuzo Sato, Makiko Yashiro, Naoki Matsuoka, Manabu Uematsu, Tomoyuki Asano, Hiroko Kobayashi, Hiroshi Watanabe, Hiromasa Ohira
Mixed connective tissue disease (MCTD) is characterized by a combination of clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis with elevated antibodies to U1 small nuclear ribonucleoprotein (U1-RNP). MCTD is often accompanied by interstitial lung disease as pulmonary involvement. On the other hand, microscopic polyangiitis (MPA) is a systemic autoimmune disease characterized by the inflammation of small vessels (arterioles, capillaries, and venules) mainly affecting the lung and kidney...
2016: Tohoku Journal of Experimental Medicine
Naoki Mugii, Minoru Hasegawa, Takashi Matsushita, Yasuhito Hamaguchi, Sacihe Oohata, Hirokazu Okita, Tetsutarou Yahata, Fujiko Someya, Katsumi Inoue, Shigeyuki Murono, Manabu Fujimoto, Kazuhiko Takehara
OBJECTIVE: Dysphagia develops with low frequency in patients with dermatomyositis. Our objective was to determine the clinical and laboratory features that can estimate the development of dysphagia in dermatomyositis. METHODS: This study included 92 Japanese patients with adult-onset dermatomyositis. The associations between dysphagia and clinical and laboratory features including disease-specific autoantibodies determined by immunoprecipitation assays were analyzed...
2016: PloS One
Cheilonda Johnson, Iago Pinal-Fernandez, Radhika Parikh, Julie Paik, Jemima Albayda, Andrew L Mammen, Lisa Christopher-Stine, Sonye Danoff
PURPOSE: Among patients with autoimmune myositis, associated interstitial lung disease (MA-ILD) is a known contributor of excess morbidity and mortality. Recent data on survival in idiopathic inflammatory myopathies originate primarily in Asia and Europe and vary widely. We sought to examine mortality in a large U.S. myositis cohort focusing in particular on the impact of associated ILD. METHODS: A cross-sectional analysis of participants from the Johns Hopkins Myositis Center with autoimmune myositis (polymyositis [PM], dermatomyositis [DM], or clinically amyopathic dermatomyositis [CADM]) was conducted...
October 2016: Lung
Hj Gayathri Devi, Md Majeed Pasha, Mantha Sathya Padmaja, Sujith Halappa
Anti-Synthetase Syndrome (ASS) is a rare autoimmune disorder characterized by Interstitial Lung Disease (ILD), inflammatory myositis, fever, Raynaud's phenomenon, mechanic's hand, and inflammatory polyarthritis in the setting of antibodies against amino acyl-transfer RNA synthetases, with anti-Jo-1 antibody being the most common. It can sometimes present as interstitial lung disease without any other expression of the syndrome. Clinical and radiological features can be similar to atypical pneumonia and could be a challenge to diagnose at an early stage...
March 2016: Journal of Clinical and Diagnostic Research: JCDR
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
Charles Sharp, Melanie McCabe, Nick Dodds, Anthony Edey, Lloyd Mayers, Huzaifa Adamali, Ann B Millar, Harsha Gunawardena
OBJECTIVE: CTD-associated interstitial lung disease (ILD) often fails to respond to conventional immunomodulatory agents. There is now considerable interest in the use of rituximab in systemic autoimmune CTD in patients refractory to standard treatments. The aim of this study was to review the experience of North Bristol NHS Trust managing patients with CTD-associated ILD with rituximab and explore possible associations with treatment response. METHODS: We conducted a retrospective analysis of all patients who received rituximab under the Bristol CTD-ILD service, having failed to respond to other immunomodulatory treatments...
July 2016: Rheumatology
Katalin Szabó, Melinda Nagy-Vincze, Levente Bodoki, Katalin Hodosi, Katalin Dankó, Zoltán Griger
INTRODUCTION: In idiopathic inflammatory myopathies, the presence of anti-Jo-1 antibody defines a distinct clinical phenotype (myositis, arthritis, interstitial lung disease, Raynaud's phenomenon fever, mechanic's hands), called antisynthetase syndrome. AIM: To determine the demographic data as well as clinical, laboratory and terapeutical features of anti-Jo1 positive patients, followed by the department of the authors. METHOD: The medical records of 49 consecutive anti-Jo1 patients were reviewed...
April 10, 2016: Orvosi Hetilap
Yizhi Xiao, Xiaoxia Zuo, Yunhui You, Hui Luo, Liping Duan, Weiru Zhang, Yisha Li, Yanli Xie, Yaou Zhou, Wangbin Ning, Tong Li, Sijia Liu, Honglin Zhu, Ying Jiang, Siyao Wu, Hongjun Zhao
Idiopathic inflammatory myopathy (IIM) is an autoimmune disease characterized by chronic muscle weakness and myositis with unknown etiology. IIM may affect the function of multiple organs and has a poor prognosis. In the present study, the causes of mortality in patients with IIM admitted to the Xiangya Hospital during the last 14 years were investigated. The investigation included an analysis of frequent causes of IIM, and of infections and associated complications. A cohort study was conducted on 676 patients with IIM that were admitted to Xiangya Hospital from January, 2001 to January, 2015...
March 2016: Experimental and Therapeutic Medicine
Cord Sunderkötter, Alexander Nast, Margitta Worm, Reinhard Dengler, Thomas Dörner, Horst Ganter, Reinhard Hohlfeld, Arthur Melms, Nico Melzer, Kai Rösler, Jens Schmidt, Michael Sinnreich, Maggi C Walter, Julia Wanschitz, Heinz Wiendl
The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup...
March 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Sandra Chartrand, Jeffrey J Swigris, Lina Peykova, Jonathan Chung, Aryeh Fischer
INTRODUCTION: Interstitial lung disease (ILD) is 1 possible manifestation of the idiopathic inflammatory myopathies (IIM). Occasionally, patients presenting with ILD are mistakenly diagnosed with idiopathic interstitial pneumonia (IIP), but after multidisciplinary evaluation, their ILD is determined to be because of antisynthetase syndrome (SynS) or myositis spectrum of disease. METHODS: We used retrospective analytic methods to identify patients with ILD evaluated at the National Jewish Health between February 2008 and August 2014 and believed initially to have IIP but ultimately diagnosed with SynS or myositis spectrum of disease...
May 2016: Journal of Rheumatology
Teck Choon Tan, Louise Wienholt, Stephen Adelstein
BACKGROUND: There is increasing recognition of a clinico-serological correlation between the idiopathic inflammatory myopathies and myositis-specific autoantibodies (MSA). We review the use of a line immunoassay-based myositis panel incorporating both MSA and myositis-associated autoantibodies (MAA) in a selected population of patients. METHODS: A retrospective analysis of patients with myositis panel assays performed in 2013 were reviewed and compared against clinical diagnoses...
December 1, 2015: International Journal of Rheumatic Diseases
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