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https://www.readbyqxmd.com/read/29127544/a-brazilian-family-with-inclusion-body-myopathy-associated-with-paget-s-disease-of-bone-and-frontotemporal-dementia-linked-to-the-vcp-pgly97glu-mutation
#1
REVIEW
Samuel Katsuyuki Shinjo, Sueli Mieko Oba-Shinjo, Antonio Marcondes Lerario, Suely Kazue Nagahashi Marie
The objective of this study is to report a Brazilian patient and his family with inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD). A systematic review of the literature on the valosin-containing protein (VCP) mutation was also performed. The proband (patient) was initially treated as a case of possible refractory polymyositis with Paget's disease and later as an inclusion body myopathy. However, after admission to our service, and considering his personal and familial antecedents, whole exome sequencing was performed revealing valosin-containing protein (VCP) c...
November 10, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29071588/a-retrospective-medical-record-review-of-utilization-patterns-and-medical-resource-use-associated-with-repository-corticotropin-injection-among-patients-with-rheumatologic-diseases-in-the-united-states
#2
Winnie W Nelson, Michael J Philbin, Jack R Gallagher, Kylee Heap, Susan Carroll, George J Wan
INTRODUCTION: Repository corticotropin injection (RCI) has anti-inflammatory and immune-modulatory effects and is approved for multiple indications, including several rheumatologic conditions. The aims of this nationally representative, retrospective, observational study were to describe patient characteristics, RCI treatment patterns, and barriers to RCI use in patients with rheumatologic disease, and to compare medical resource use (MRU) before and after RCI therapy. METHODS: A random sample of US physicians was recruited to abstract the medical records of deidentified patients with a diagnosis of rheumatoid arthritis (RA), psoriatic arthritis (PsA), dermatomyositis/polymyositis (DM/PM), or systemic lupus erythematosus (SLE) who had been treated with RCI in the previous 24 months...
October 25, 2017: Rheumatology and Therapy
https://www.readbyqxmd.com/read/29051891/scleroderma-renal-crisis-in-mixed-connective-tissue-disease-with-full-renal-recovery-within-3-months-a-case-report-with-expanding-treatment-modalities-to-treat-each-clinical-sign-as-an-independent-entity
#3
Jordana Cheta, Suresh Rijhwani, Harlan Rust
Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29050128/-lung-transplantation-for-connective-tissue-disease-associated-interstitial-lung-disease-recipient
#4
J Zhang, B Wu, M Zhou, D Liu, L Fan, D Wei, H Yang, J Y Chen
Objective: To investigate the timing and complications of lung transplantation for the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD). Method: The clinical data of connective tissue-associated disease lung transplantation recipients from September, 2015 to February, 2017 were collected. Results: 11 patients with CTD-ILD were evaluated by lung transplantation and were treated with lung transplantation. Including 2 cases dermatomyositis/polymyositis and interstitial lung disease (PM/DM-ILD), 4 cases rheumatoid arthritis and interstitial lung disease (RA-ILD), 4 cases of primary Sjgren's syndrome and interstitial lung disease (pSS-ILD), 1 case of systemic sclerosis and interstitial lung disease (SSc-ILD)...
October 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29027009/lipid-lowering-agent-triggered-dermatomyositis-and-polymyositis-a-case-series-and-literature-review
#5
REVIEW
Isabela Bruna Pires Borges, Marilda Guimarães Silva, Rafael Giovane Misse, Samuel Katsuyuki Shinjo
Lipid-lowering agent-triggered dermatomyositis (DM) or polymyositis (PM) is a rare event. Therefore, the aim of the present study was to describe a series of such cases. A retrospective cohort study of 5 DM and 4 PM cases triggered by prior exposure to lipid-lowering agents between 2001 and 2017 was carried out. All patients, except for two cases, had muscle biopsy compatible with inflammatory myopathy and no serum autoantibodies positive for anti-SRP or anti-HMGCoAR. Median age of the patients at time of diagnosis was 68 years...
October 12, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28994016/cardiopulmonary-manifestations-of-collagen-vascular-diseases
#6
REVIEW
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28993346/abatacept-in-the-treatment-of-adult-dermatomyositis-and-polymyositis-a-randomised-phase-iib-treatment-delayed-start-trial
#7
Anna Tjärnlund, Quan Tang, Cecilia Wick, Maryam Dastmalchi, Herman Mann, Jana Tomasová Studýnková, Radka Chura, Nicola J Gullick, Rosaria Salerno, Johan Rönnelid, Helene Alexanderson, Eva Lindroos, Rohit Aggarwal, Patrick Gordon, Jiri Vencovsky, Ingrid E Lundberg
OBJECTIVES: To study the effects of abatacept on disease activity and on muscle biopsy features of adult patients with dermatomyositis (DM) or polymyositis (PM). METHODS: Twenty patients with DM (n=9) or PM (n=11) with refractory disease were enrolled in a randomised treatment delayed-start trial to receive either immediate active treatment with intravenous abatacept or a 3 month delayed-start. The primary endpoint was number of responders, defined by the International Myositis Assessment and Clinical Studies Group definition of improvement (DOI), after 6 months of treatment...
October 9, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28977549/2016-acr-eular-adult-dermatomyositis-and-polymyositis-and-juvenile-dermatomyositis-response-criteria-methodological-aspects
#8
Lisa G Rider, Nicolino Ruperto, Angela Pistorio, Brian Erman, Nastaran Bayat, Peter A Lachenbruch, Howard Rockette, Brian M Feldman, Adam M Huber, Paul Hansen, Chester V Oddis, Ingrid E Lundberg, Anthony A Amato, Hector Chinoy, Robert G Cooper, Lorinda Chung, Katalin Danko, David Fiorentino, Ignacio García-De la Torre, Ann M Reed, Yeong Wook Song, Rolando Cimaz, Rubén J Cuttica, Clarissa A Pilkington, Alberto Martini, Janjaap van der Net, Susan Maillard, Frederick W Miller, Jiri Vencovsky, Rohit Aggarwal
Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM. Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions...
November 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28975463/risk-factors-for-the-recurrence-of-interstitial-lung-disease-in-patients-with-polymyositis-and-dermatomyositis-a-retrospective-cohort-study
#9
Maho Nakazawa, Yuko Kaneko, Tsutomu Takeuchi
To identify risk factors for the recurrence of interstitial lung disease (ILD) in patients with polymyositis (PM)/dermatomyositis (DM). Forty-four PM/DM-ILD patients who had been treated with glucocorticoid and/or immunosuppressive agents as a remission induction therapy were enrolled. The patients were first classified into two groups: the early recurrence group that recurred within 52 weeks, and the non-early recurrence group, which was further classified into the late recurrence group that recurred after 52 weeks, and the non-recurrence group...
October 3, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28973843/comment-on-the-article-titled-increased-incidence-of-amyotrophic-lateral-sclerosis-in-polymyositis-a-nationwide-cohort-study
#10
Konstantinos Parperis
With interest, I read the recent article in Arthritis Care and Research titled "Increased Incidence of Amyotrophic Lateral Sclerosis in Polymyositis: A Nationwide Cohort Study" (1). Tseng at al (1) conducted a retrospective cohort study in Taiwan, exploring a link between amyotrophic lateral sclerosis (ALS) and polymyositis (PM). This article is protected by copyright. All rights reserved.
October 3, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28973839/reply
#11
Chia-Chun Tseng, Shun-Jen Chang, Wen-Chan Tsai, Tsan-Teng Ou, Cheng-Chin Wu, Wan-Yu Sung, Ming-Chia Hsieh, Jeng-Hsien Yen
We appreciate the comments from Dr Parperis. The question raised by Parperis was the possibility of amyotrophic lateral sclerosis (ALS) misdiagnosed as polymyositis (PM) initially. Dr Parperis cited the study of Harrington et al to support his claims (1). However, several points had to be considered. This article is protected by copyright. All rights reserved.
October 3, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28942404/successful-treatment-using-rituximab-in-a-patient-with-refractory-polymyositis-complicated-by-scleroderma-renal-crisis
#12
Kyoko Innami, Tomoyuki Mukai, Shoko Kodama, Yoshitaka Morita
Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab...
September 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28931462/paradoxical-development-of-polymyositis-like-autoimmunity-through-augmented-expression-of-autoimmune-regulator-aire
#13
Hitoshi Nishijima, Tatsuya Kajimoto, Yoshiki Matsuoka, Yasuhiro Mouri, Junko Morimoto, Minoru Matsumoto, Hiroshi Kawano, Yasuhiko Nishioka, Hisanori Uehara, Keisuke Izumi, Koichi Tsuneyama, Il-Mi Okazaki, Taku Okazaki, Kazuyoshi Hosomichi, Ayako Shiraki, Makoto Shibutani, Kunitoshi Mitsumori, Mitsuru Matsumoto
Autoimmunity is prevented by the function of the autoimmune regulator [AIRE (Aire in mice)], which promotes the expression of a wide variety of tissue-restricted antigens (TRAs) from medullary thymic epithelial cells (mTECs) and from a subset of peripheral antigen-presenting cells (APCs). We examined the effect of additive expression of human AIRE (huAIRE) in a model of autoimmune diabetes in NOD mice. Unexpectedly, we observed that mice expressing augmented AIRE/Aire developed muscle-specific autoimmunity associated with incomplete maturation of mTECs together with impaired expression of Aire-dependent TRAs...
September 17, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28928949/plasmapheresis-in-neurological-disorders-six-years-experience-from-university-clinical-center-tuzla
#14
Osman Sinanović, Sanela Zukić, Adnan Burina, Nermina Pirić, Renata Hodžić, Mirza Atić, Mirna Alečković-Halilović, Enisa Mešić
BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this therapy...
2017: F1000Research
https://www.readbyqxmd.com/read/28926465/extensive-dystrophic-calcinosis-cutis-a-severe-complication-of-polymyositis-systemic-sclerosis-overlap-syndrome
#15
Simon Smith, Josh Hanson
No abstract text is available yet for this article.
September 19, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28893408/inflammatory-myopathy-associated-with-antimitochondrial-antibodies-a-distinct-phenotype-with-cardiac-involvement
#16
Jemima Albayda, Aamna Khan, Livia Casciola-Rosen, Andrea M Corse, Julie J Paik, Lisa Christopher-Stine
OBJECTIVE: In the context of clinical evaluations performed on our prospective myositis cohort, we noted a striking association of severe cardiac disease in myositis patients with antimitochondrial antibodies. We sought to review all cases of antimitochondrial antibody (AMA) associated myositis in our cohort to describe the clinical features of this disease subset. METHODS: We identified 7 patients with confirmed antimitochondrial antibodies who presented as an inflammatory myopathy...
June 13, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28879041/pulmonary-arterial-hypertension-a-novelty-in-idiopathic-inflammatory-myopathies-insights-and-first-experiences-with-vasoactive-therapy
#17
Kavish J Bhansing, Anton Vonk-Noordegraaf, Frank Pt Oosterveer, Piet Lcm van Riel, Madelon C Vonk
To characterise the different types of pulmonary hypertension (PH) among idiopathic inflammatory myopathy (IIM). A retrospective case series with assessment of PH by right heart catheterisation, extent of interstitial lung disease (ILD) and outcome of vasoactive therapy.The group of patients with IIM with PH (n=9) showed a median age at PH diagnosis of 62 years (IQR 48-71 years; eight women), seven diagnosed with polymyositis and two with dermatomyositis; median disease duration of 5.7 years and five patients with a positive anti-Jo1 antibody...
2017: RMD Open
https://www.readbyqxmd.com/read/28872085/-manifestations-of-the-connective-tissue-associated-interstitial-lung-disease-under-high-resolution-computed-tomography
#18
Hailan Li, Zeng Xiong, Jinkang Liu, Yisha Li, Bin Zhou
To analyze the features of the connective tissue associated interstitial lung disease (CTD-ILD) by high resolution computed tomography (HRCT).
 Methods: A total of 127 patients with CTD-ILD, who were diagnosed by clinic laboratory examination and pathology in Xiangya Hospital of Central South University form September 2013 to September 2015, were enrolled for this study. Their lung features of HRCT imaging were retrospectively analyzed.
 Results: The classifications for 127 patients were as follows: 36 cases of rheumatoid arthritis (28...
August 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28869080/altered-chemokine-receptor-expression-in-the-peripheral-blood-lymphocytes-in-polymyositis-and-dermatomyositis
#19
Ziwei Zhu, Chunshu Yang, Jianing Wang, Qianhui Feng, Qi Chen, Pingting Yang
OBJECTIVE: To examine the expression of chemokine receptors in different peripheral blood T-cell subsets in patients with polymyositis (PM) and dermatomyositis (DM). METHODS: We used flow cytometry to measure the frequencies of chemokinereceptors CXCR3 and CCR4 expression in the CD4(+) or CD8(+) lymphocytes. Enzyme linked immunosorbent assays were also used to measure the concentrations of C-X-C motif chemokine 10 (CXCL10), thymus and activation regulated chemokine (TARC) and macrophage derived chemokine (MDC)...
August 28, 2017: Cytokine
https://www.readbyqxmd.com/read/28866745/mortality-and-prognostic-factors-in-idiopathic-inflammatory-myositis-a-retrospective-analysis-of-a-large-multicenter-cohort-of-spain
#20
Laura Nuño-Nuño, Beatriz Esther Joven, Patricia E Carreira, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente Cubas, Eva Gloria Tomero, María Carmen Barbadillo-Mateos, Paloma García De la Peña Lefebvre, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, Henry Moruno-Cruz, Ana Pérez, Tatiana Cobo-Ibáñez, Raquel Almodóvar González, Leticia Lojo, María Jesús García De Yébenes, Francisco Javier López-Longo
The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9...
November 2017: Rheumatology International
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