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Camptocormia with Anti Ku antibodies in Inclusion Body Myositis (P2.112)
Conclusions:This case shows the first published case anti – ku antibodies present in IBM in association scleroderma with camptocormia. The definitive pathology and presentation were classic for IBM with exception of the camptocormia but does fit in scleroderma with anti-ku antibodies. Given the autoimmune component of her disease, patient was started on immunosuppressive medication which stabilized her physical exam.Disclosure: Dr. Jens has nothing to disclose. Dr. Kovacik Eicher has nothing to disclose. Dr. Kaur has nothing to disclose. (Source: Neurology)
Source: Neurology - April 17, 2017 Category: Neurology Authors: Jens, W., Eicher, M. E. K., Kaur, D. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Intervention Effect on Fellow Muscle Biopsy Reading Accuracy in Inflammatory Myositis (P2.124)
Conclusions:Improved accuracy in the interpretation of IIM pathology amongst inexperienced muscle biopsy readers can support the use of a limited standardized educational program.Disclosure: Dr. Hamasaki has nothing to disclose. Dr. Jawdat has nothing to disclose. Dr. Kimple has nothing to disclose. Dr. Dimachkie has received personal compensation for activities with Pfizer, Depomed, Merck, CSL-Behring, Nufactor, Biomarin, Baxter and Catalyst as a consultant and/or speaker. Dr. Pasnoor has nothing to disclose. Dr. Statland has received personal compensation for activities with Sarepta, ATYR Bristol Meyers Squib, Acceleron,...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Hamasaki, A., Jawdat, O., Kimple, D., Dimachkie, M., Pasnoor, M., Statland, J., Barohn, R., Glenn, M., Herbelin, L. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Performance test evaluation of intra-rater and inter-rater reliability among examiners of histopathological findings in myositis subtypes (P2.132)
Conclusions:This study explores intra and inter reliability for diagnosing DM, PM and NM by muscle biopsy specimens. If consistency can be shown in muscle biopsy interpretation by studying intra-rater and inter-rater reliability in fellows studying neuromuscular medicine, this will help to validate the use of muscle biopsy for diagnostic use in medical professionals with limited training.Disclosure: Dr. Kimple has nothing to disclose. Dr Barohn received personal compensation for activities with Grifols & Genzyme as a member of the Speakers Bureau and NuFactor as a consultant. Dr. Dimachkie has received personal compens...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Kimple, D., Barohn, R., Dimachkie, M., Pasnoor, M., Glenn, M., Herbelin, L., Statland, J., Jawdat, O. Tags: Myopathies & amp;amp; Myasthenia Gravis II Source Type: research

Mitochondrial Skeletal and Cardiac Myopathy Responsive to Aggressive Immunosuppression in Rapidly Progressing Systemic Sclerosis (P5.077)
Conclusions:Perivascular epimysial inflammation is rarely seen in any disease. Mitochondrial myopathy without myositis has not been reported in systemic sclerosis. We propose that ischemia caused by vasculopathy and fibrosis in systemic sclerosis, together with subclinical mitochondrial pathology, severely affected skeletal and cardiac muscle due to their high metabolic demands. An unusual presentation of myopathy in autoimmune disease suggests an underlying latent pathology, such as mitochondrial myopathy. Rapidly progressing autoimmune disease necessitates aggressive immunosuppression.Disclosure: Dr. Yeo has nothing to d...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Yeo, C., Bunin, V., Jovan, P., Cykowski, M., Smith, R. Tags: Neuromuscular and Clinical Neurophysiology (EMG) ePoster Session Source Type: research

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies
Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. (Source: Respiratory Medicine)
Source: Respiratory Medicine - April 15, 2017 Category: Respiratory Medicine Authors: Kiminobu Tanizawa, Tomohiro Handa, Ran Nakashima, Takeshi Kubo, Yuji Hosono, Kizuku Watanabe, Kensaku Aihara, Kohei Ikezoe, Akihiko Sokai, Yoshinari Nakatsuka, Yoshio Taguchi, Kazuhiro Hatta, Satoshi Noma, Yoichiro Kobashi, Akihiko Yoshizawa, Toru Oga, To Source Type: research

Inflammatory myopathy associated with myasthenia gravis with and without thymic pathology: Report of four cases and literature review
Discussion the recognition of these neuromuscular co-morbidities contributes to (i) understanding their pathogenic mechanisms, (ii) developing better management approaches and (iii) further improving disease outcomes. (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - April 14, 2017 Category: Allergy & Immunology Source Type: research

Toll-Like Receptor 4 –Myeloid Differentiation Primary Response Gene 88 Pathway Is Involved in the Inflammatory Development of Polymyositis by Mediating Interferon-γ and Interleukin-17A in Humans and Experimental Autoimmune Myositis Mouse Model
Hongya Zhang, Fangyuan He, Ming Shi, Wenxiu Wang, Xiaojia Tian, Juan Kang, Wenjuan Han, Rui Wu, Linfu Zhou, Mengmeng Hu, Xiaobo Li, Fang Mi, Gang Zhao, Hongge Jia (Source: Frontiers in Neurology)
Source: Frontiers in Neurology - April 12, 2017 Category: Neurology Source Type: research

401 Cancer risk in clinically amyopathic dermatomyositis: A retrospective cohort study at four tertiary care centers
Clinically amyopathic dermatomyositis (CADM), characterized by pathognomonic cutaneous findings without muscle weakness, is an important subset and accounts for 20% of patients with dermatomyositis (DM). In patients with CADM, limited literature exists regarding the associated risk of malignancy as most studies have focused on classic DM or polymyositis. Therefore, we investigated the association between CADM and cancer at 4 tertiary care centers. Using the Partners Healthcare and New York University medical record systems, we reviewed the medical records of all patients with CADM treated between 2000 and 2016. (Source: Jo...
Source: Journal of Investigative Dermatology - April 12, 2017 Category: Dermatology Authors: J. Pinard, M. Roman, D. Kurtzman, A. Ho, A. Femia, R. Vleugels Tags: Clinical Research: Patient Outcomes Research Source Type: research

Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients
Conclusions: HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies. (Source: Neurology)
Source: Neurology - April 10, 2017 Category: Neurology Authors: Lloyd, T. E., Pinal-Fernandez, I., Michelle, E. H., Christopher-Stine, L., Pak, K., Sacktor, N., Mammen, A. L. Tags: HIV, Muscle disease ARTICLE Source Type: research

2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
To develop response criteria for juvenile dermatomyositis (DM). We analysed the performance of 312 definitions that used core set measures from either the International Myositis Assessment and Clinical Studies Group (IMACS) or the Paediatric Rheumatology International Trials Organisation (PRINTO) and were derived from natural history data and a conjoint analysis survey. They were further validated using data from the PRINTO trial of prednisone alone compared to prednisone with methotrexate or cyclosporine and the Rituximab in Myositis (RIM) trial. At a consensus conference, experts considered 14 top candidate criteria base...
Source: Annals of the Rheumatic Diseases - April 6, 2017 Category: Rheumatology Authors: Rider, L. G., Aggarwal, R., Pistorio, A., Bayat, N., Erman, B., Feldman, B. M., Huber, A. M., Cimaz, R., Cuttica, R. J., de Oliveira, S. K., Lindsley, C. B., Pilkington, C. A., Punaro, M., Ravelli, A., Reed, A. M., Rouster-Stevens, K., van Royen-Kerkhof, Tags: Criteria Source Type: research

2016 American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate, and major clinical response in adult dermatomyositis and polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM). Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined the relative weights of core set measures and conjoint analysis definitions. The performance characteristics of the definitions were evaluated on p...
Source: Annals of the Rheumatic Diseases - April 6, 2017 Category: Rheumatology Authors: Aggarwal, R., Rider, L. G., Ruperto, N., Bayat, N., Erman, B., Feldman, B. M., Oddis, C. V., Amato, A. A., Chinoy, H., Cooper, R. G., Dastmalchi, M., Fiorentino, D., Isenberg, D., Katz, J. D., Mammen, A., de Visser, M., Ytterberg, S. R., Lundberg, I. E., Tags: Criteria Source Type: research

2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Juvenile Dermatomyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
ConclusionThe response criteria for juvenile DM consisted of a conjoint analysis–based model using a continuous improvement score based on absolute percent change in core set measures, with thresholds for minimal, moderate, and major improvement. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - April 6, 2017 Category: Rheumatology Authors: Lisa G. Rider, Rohit Aggarwal, Angela Pistorio, Nastaran Bayat, Brian Erman, Brian M. Feldman, Adam M. Huber, Rolando Cimaz, Rub én J. Cuttica, Sheila Knupp de Oliveira, Carol B. Lindsley, Clarissa A. Pilkington, Marilynn Punaro, Angelo Ravelli, Ann M. R Tags: Special Article Source Type: research

2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
ConclusionThe response criteria for adult DM/PM consisted of the conjoint analysis model based on absolute percent change in 6 core set measures, with thresholds for minimal, moderate, and major improvement. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - April 6, 2017 Category: Rheumatology Authors: Rohit Aggarwal, Lisa G. Rider, Nicolino Ruperto, Nastaran Bayat, Brian Erman, Brian M. Feldman, Chester V. Oddis, Anthony A. Amato, Hector Chinoy, Robert G. Cooper, Maryam Dastmalchi, David Fiorentino, David Isenberg, James D. Katz, Andrew Mammen, Mariann Tags: Special Article Source Type: research

Subclinical Cardiac Dysfunction in Polymyositis and Dermatomyositis: A Speckle-tracking Case-control Study.
CONCLUSION: Subclinical systolic impairment is common in patients with IIM without overt LV dysfunction. In this context, GLS is a potentially useful variable. PMID: 28365571 [PubMed - as supplied by publisher] (Source: Journal of Rheumatology)
Source: Journal of Rheumatology - April 4, 2017 Category: Rheumatology Tags: J Rheumatol Source Type: research

High ‐resolution manometry in patients with idiopathic inflammatory myopathy: Elevated prevalence of esophageal involvement and differences according to autoantibody status and clinical subset
ConclusionsEsophageal involvement is common in myositis patients, but it correlates poorly with esophageal symptoms. Specific clinical and serologic groups have different manometric features. Muscle Nerve, 2017 (Source: Muscle and Nerve)
Source: Muscle and Nerve - April 4, 2017 Category: Internal Medicine Authors: Maria Casal ‐Dominguez, Iago Pinal‐Fernandez, Marianela Mego, Anna Accarino, Lluis Jubany, Fernando Azpiroz, Albert Selva‐O'callaghan Tags: Research Article Source Type: research

Immune ‐Array Analysis in Sporadic Inclusion Body Myositis Reveals HLA–DRB1 Amino Acid Heterogeneity Across the Myositis Spectrum
ConclusionThis is the largest, most comprehensive genetic association study to date in IBM. The data confirm that HLA is the most strongly associated region and identifies novel amino acid associations that may explain the risk in this locus. These amino acid associations differentiate IBM from polymyositis and dermatomyositis and may determine properties of the peptide‐binding groove, allowing it to preferentially bind autoantigenic peptides. A novel suggestive association within the chromosome 3 p21.31 region suggests a role for CCR5. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - April 4, 2017 Category: Rheumatology Authors: Simon Rothwell, Robert G. Cooper, Ingrid E. Lundberg, Peter K. Gregersen, Michael G. Hanna, Pedro M. Machado, Megan K. Herbert, Ger J. M. Pruijn, James B. Lilleker, Mark Roberts, John Bowes, Michael F. Seldin, Jiri Vencovsky, Katalin Danko, Vidya Limaye, Tags: Full Length Source Type: research

DEPTOR-mTOR Signaling Is Critical for Lipid Metabolism and Inflammation Homeostasis of Lymphocytes in Human PBMC Culture.
Authors: Xie QB, Liang Y, Yang M, Yang Y, Cen XM, Yin G Abstract Abnormal immune response of the body against substances and tissues causes autoimmune diseases, such as polymyositis, dermatomyositis, and rheumatoid arthritis. Irregular lipid metabolism and inflammation may be a significant cause of autoimmune diseases. Although much progress has been made, mechanisms of initiation and proceeding of metabolic and inflammatory regulation in autoimmune disease have not been well-defined. And novel markers for the detection and therapy of autoimmune disease are urgent. mTOR signaling is a central regulator of extracell...
Source: Journal of Immunology Research - March 31, 2017 Category: Allergy & Immunology Tags: J Immunol Res Source Type: research

Cardiac transplantation in dermatomyositis: A case report and literature review
Conclusions Severe cardiac involvement requiring transplantation is rare in dermatomyositis but does occur and appears to be related to a similar inflammatory process as noted in the skeletal muscle. (Source: Human Pathology: Case Reports)
Source: Human Pathology: Case Reports - March 31, 2017 Category: Pathology Source Type: research

[Polymyositis secondary to a non-Hodgkin's lymphoma].
PMID: 28345740 [PubMed - in process] (Source: Revista de Neurologia)
Source: Revista de Neurologia - March 29, 2017 Category: Neurology Authors: Gil-Moreno MJ, Escolar E, Marasescu R, Camacho-Castaneda FI, Benito-Parra L Tags: Rev Neurol Source Type: research

A case of anti-aminoacyl tRNA synthetase (ARS) antibody-positive polymyositis (PM)/dermatomyositis (DM)-associated interstitial pneumonia (IP) successfully controlled with bosentan therapy
Publication date: Available online 29 March 2017 Source:Respiratory Medicine Case Reports Author(s): Tomoyuki Naito, Yosuke Tanaka, Mitsunori Hiron, 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)...
Source: Respiratory Medicine Case Reports - March 29, 2017 Category: Respiratory Medicine Source Type: research

Red blood cell distribution width and neutrophil to lymphocyte ratio are correlated with disease activity of dermatomyositis and polymyositis
ConclusionBoth RDW and NLR are useful indices in assessing the disease activity of PM/DM. (Source: Journal of Clinical Laboratory Analysis)
Source: Journal of Clinical Laboratory Analysis - March 27, 2017 Category: Laboratory Medicine Authors: Ming ‐Zhu Gao, Yuan‐Lan Huang, Xiao‐Dan Wu, Qiang‐Wei Xu, Rong Ji, Bing Gu, Ai‐Yan Zhang, Ting‐Ting Hao, Zhi‐Jun Han, Tian‐Li Ren Tags: RESEARCH ARTICLE Source Type: research

Juvenile dermatomyositis/polymyositis and lymphoma
The objective of this literature review was to assess for any association between, and disease profile of, JDM/PM and lymphoma in childhood. Risk determinants of the possible development of lymphoma in the pediatric population with JDM/PM appear to be the degree and duration of inflammatory activity with chronic B-cell activation and/or antigen stimulation; a paraneoplastic relationship is unlikely. (Source: Journal of the Neurological Sciences)
Source: Journal of the Neurological Sciences - March 25, 2017 Category: Neurology Authors: Joerg-Patrick St übgen Tags: Review Article Source Type: research

Inflammatory Muscle Disease: A New Landscape
Publication date: Available online 22 March 2017 Source:Joint Bone Spine Author(s): 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 muscle diseases (IMDs) resemble inflammatory joint diseases in that they constitute a highly heterogeneous group of conditions. The topographic distribution, severity, and tempo of onset vary widely, and the histological findings distinguish at...
Source: Joint Bone Spine - March 23, 2017 Category: Orthopaedics Source Type: research

Relevance of clinical and autoantibody profiles in systemic sclerosis among Thais
ConclusionsATA and anti‐CENP were not helpful in differentiating the SSc subset in Thai SSc patients, albeit they were good for predicting hand function. Coexisting ATA and anti‐CENP negativity were associated with less extensive skin tightness and SSc overlap syndrome. (Source: APLAR Journal of Rheumatology)
Source: APLAR Journal of Rheumatology - March 9, 2017 Category: Rheumatology Authors: Chingching Foocharoen, Piyakarn Watcharenwong, Sittichai Netwijitpan, Ajanee Mahakkanukrauh, Siraphop Suwannaroj, Ratanavadee Nanagara Tags: Original Article Source Type: research

Incidence of autoimmune diseases in a nationwide HIV/AIDS patient cohort in Taiwan, 2000-2012
Conclusions PLWHA had higher risks of incident Sjögren syndrome, psoriasis, SLE, autoimmune haemolytic anaemia and uveitis. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - March 9, 2017 Category: Rheumatology Authors: Yen, Y.-F., Chuang, P.-H., Jen, I.-A., Chen, M., Lan, Y.-C., Liu, Y.-L., Lee, Y., Chen, Y.-H., Chen, Y.-M. A. Tags: Immunology (including allergy), Muscle disease, Ophthalmology, Connective tissue disease, Degenerative joint disease, Musculoskeletal syndromes, Rheumatoid arthritis, Systemic lupus erythematosus Clinical and epidemiological research Source Type: research

Thigh muscle MRI in immune-mediated necrotising myopathy: extensive oedema, early muscle damage and role of anti-SRP autoantibodies as a marker of severity
Conclusions Compared with patients with DM or PM, IMNM is characterised by more widespread muscle involvement. Anti-SRP-positive patients have more severe muscle involvement than anti-HMGCR-positive patients. (Source: Annals of the Rheumatic Diseases)
Source: Annals of the Rheumatic Diseases - March 9, 2017 Category: Rheumatology Authors: Pinal-Fernandez, I., Casal-Dominguez, M., Carrino, J. A., Lahouti, A. H., Basharat, P., Albayda, J., Paik, J. J., Ahlawat, S., Danoff, S. K., Lloyd, T. E., Mammen, A. L., Christopher-Stine, L. Tags: Immunology (including allergy), Muscle disease, Connective tissue disease, Musculoskeletal syndromes Clinical and epidemiological research Source Type: research

Neurological manifestations in dogs naturally infected by Leishmania infantum: descriptions of 10 cases and a review of the literature
This study confirms that both central and peripheral nervous systems can be affected by leishmaniasis and provides an overview on the possible etiopathogenetic mechanisms. In addition, clinical and diagnostic findings, therapy and follow‐up of affected dogs are described. (Source: The Journal of Small Animal Practice)
Source: The Journal of Small Animal Practice - March 6, 2017 Category: Veterinary Research Authors: A. P. Giannuzzi, M. Ricciardi, A. De Simone, F. Gernone Tags: REVIEW Source Type: research

The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR)
ConclusionsUsing the J-RBR database, our study revealed that biopsy-confirmed cases of connective tissue diseases such as RA, SjS, scleroderma, and MCTD show various clinical and pathological characteristics, depending on the underlying diseases and the medication used. (Source: Clinical and Experimental Nephrology)
Source: Clinical and Experimental Nephrology - March 1, 2017 Category: Urology & Nephrology Source Type: research

Increased risk of comorbid rheumatic disorders in vitiligo patients: A nationwide population ‐based study
Abstract Vitiligo is a common acquired depigmentation disorder. Previous studies have shown that vitiligo is associated with a variety of autoimmune disorders. However, a large‐scale epidemiological study focused on comorbid rheumatic disorders has not been undertaken. To clarify the associations between vitiligo and various rheumatic disorders, we performed a cross‐sectional study using data from the Korean National Health Insurance claims database. Between 2009 and 2013, totals of 86 210 patients with vitiligo and 172 420 age‐ and sex‐matched controls without vitiligo were enrolled in this study. Vitiligo patient...
Source: The Journal of Dermatology - March 1, 2017 Category: Dermatology Authors: Chong Won Choi, Sung Hye Eun, Kwang Hyun Choi, Jung Min Bae Tags: Original Article Source Type: research

Successful treatment of a refractory dysbiotic intestinal pseudo-obstruction in a patient with systemic sclerosis-polymyositis overlap syndrome by intravenous immunoglobulin administration possibly related to gut flora normalisation.
Authors: Kamei R, Yamaoka T, Ikinaga K, Murota H, Shimizu K, Katayama I Abstract ****************************************************************************. PMID: 28229828 [PubMed - as supplied by publisher] (Source: Clinical and Experimental Rheumatology)
Source: Clinical and Experimental Rheumatology - February 25, 2017 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research

Effective Use of Calcineurin Inhibitor in Combination Therapy for Interstitial Lung Disease in Patients With Dermatomyositis and Polymyositis
Objective: The current study demonstrated the potential use of calcineurin inhibitor (CNI) in combination therapy for interstitial lung disease (ILD) complicated with dermatomyositis (DM) and polymyositis (PM). Methods: Thirty DM/PM patients with ILD were enrolled in this study. Continuous intravenous administration of cyclosporine A (IV-CsA) was simultaneously started with corticosteroid in patients presenting more than two respiratory distress factors as follows: (Source: JCR: Journal of Clinical Rheumatology)
Source: JCR: Journal of Clinical Rheumatology - February 23, 2017 Category: Rheumatology Tags: Original Articles Source Type: research

Effects of exercise therapy on polymyositis complicated by post-myocarditis cardiomyopathy: A case report.
CONCLUSION: With appropriate care to avoid exacerbation of heart failure and myositis, staged increases in the volume of supervised and unsupervised exercise therapy can safely and effectively maintain and improve physical capacity, exercise tolerance, and overall physical activity. PMID: 28218333 [PubMed - as supplied by publisher] (Source: Journal of Rehabilitation Medicine)
Source: Journal of Rehabilitation Medicine - February 23, 2017 Category: Rehabilitation Tags: J Rehabil Med Source Type: research

Isolated Ro52 Antibodies as Immunological Marker of a Mild Phenotype of Undifferentiated Connective Tissue Diseases.
The objective of this retrospective study was to investigate if there was a milder subgroup of UCTD with a special clinical profile consisting only in the presence of anti-Ro52 autoantibodies. Immunological and clinical records of 62 patients attending the hospital during 30 months were studied. Results showed a target population formed by mostly women, aged between 40 and 80 years at the moment of the study, with a registered age of onset between 40 and 60 years. Speckled pattern was the most frequent pattern found by indirect immunofluorescence. Given the obtained results and keeping in mind possible limitations because ...
Source: International Journal of Rheumatology - February 18, 2017 Category: Rheumatology Tags: Int J Rheumatol Source Type: research

Identification of Palmitoleic Acid Controlled by mTOR Signaling as a Biomarker of Polymyositis.
In this study, we aim to figure out the role of mTOR signaling in the development of polymyositis and identify novel biomarkers for the detection and therapy of polymyositis. After screening and validation, we found that palmitoleic acid, a monounsaturated fatty acid, is highly regulated by mTOR signaling. Inhibition of mTORC1 activity decreases palmitoleic acid level. Moreover, mTORC1 regulates the level of palmitoleic acid by controlling its de novo synthesis. Importantly, increased palmitoleic acid has been proven to be a marker of polymyositis. Our work identifies palmitoleic acid in peripheral blood mononuclear cells ...
Source: Journal of Immunology Research - February 16, 2017 Category: Allergy & Immunology Tags: J Immunol Res Source Type: research

Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases
Conclusions Patients with PM/DM/CADM-ILD are at risk for chronic respiratory failure due to the deterioration of ILD during long-term follow-up. The presence of anti-PL-7 antibody and a lower %FVC at initial diagnosis may predict long-term deterioration in patients with PM/DM/CADM-ILD. (Source: Respiratory Investigation)
Source: Respiratory Investigation - February 8, 2017 Category: Respiratory Medicine Source Type: research

A study of acute muscle dysfunction with particular reference to dengue myopathy
Conclusion: Dengue infection and hypokalemia due to various causes are the most common causes of acute myopathy and are associated with rapid and complete recovery within 1 month. Shorter duration of illness, higher MRC sum score, better disability status at presentation, lower serum CK correlate with better outcome. Biopsy was decisive in <20% cases; hence, it is not primary investigation in acute myopathy. (Source: Annals of Indian Academy of Neurology)
Source: Annals of Indian Academy of Neurology - February 8, 2017 Category: Neurology Authors: Rajesh Verma Vikram V Holla Vijay Kumar Amita Jain Nuzhat Husain Kiran Preet Malhotra Ravindra Kumar Garg Hardeep Singh Malhotra Praveen Kumar Sharma Neeraj Kumar Source Type: research

Association between rheumatic diseases and cancer: results from a clinical practice cohort study
AbstractThe association between cancer and immune-mediated rheumatic conditions is controversial, especially as far as polymyalgia rheumatica (PMR) is concerned. Furthermore, no clinical feature has been shown to be suggestive of a paraneoplastic rheumatic syndrome. With the present study, we aim to address both these issues. The study population comprisedN = 1750 patients, includingN = 100 with PMR, who attended our tertiary immuno-rheumatology clinic between January 1, 2005 and November 30, 2012. A rheumatic disease was deemed paraneoplastic if cancer had been diagnosed in the 2 years preceding or following its onse...
Source: Internal and Emergency Medicine - February 7, 2017 Category: Emergency Medicine Source Type: research

Hepatitis C virus and human T-cell lymphotropic virus type 1 co-infection: impact on liver disease, virological markers, and neurological outcomes
Human T-cell lymphotropic virus type 1 (HTLV-1) infects 5 –10 million people worldwide and is endemic in Japan, the Caribbean, Sub-Saharan Africa, the Middle East, and South America, particularly in Brazil.1 HTLV-1 causes a persistent infection of CD4+ and CD8+ T-cells that leads to diverse changes in the immune response.2 It can be associated with the d evelopment of adult T-cell leukemia/lymphoma (ATLL) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP).3 In addition to its pathogenic role in these diseases, HTLV-1 has also been associated with other immune-mediated conditions, such as uveitis, pol...
Source: International Journal of Infectious Diseases - February 5, 2017 Category: Infectious Diseases Authors: Ot ávio M. Espíndola, Alexandre G. Vizzoni, Elisabeth Lampe, Maria José Andrada-Serpa, Abelardo Q.C. Araújo, Ana Claudia C. Leite Source Type: research

Myalgia in Patients with Dermatomyositis and Polymyositis Is Attributable to Fasciitis Rather Than Myositis: A Retrospective Study of 32 Patients who Underwent Histopathological Examinations.
CONCLUSION: The frequency of fasciitis was significantly higher among patients with DM than among those with PM. Fasciitis, rather than myositis, was associated with myalgia. PMID: 28148694 [PubMed - as supplied by publisher] (Source: Journal of Rheumatology)
Source: Journal of Rheumatology - February 4, 2017 Category: Rheumatology Tags: J Rheumatol Source Type: research

The Correlation of Muscle Biopsy Scores with the Clinical Variables in Idiopathic Inflammatory Myopathies.
CONCLUSION: In this study, muscle biopsy cannot be used to differentiate among subgroups of IIM patients. In addition, we found only modest correlation of muscle biopsy scores with muscle power and CK. Further study is necessary to confirm our findings. PMID: 28144368 [PubMed - in process] (Source: Open Rheumatology Journal)
Source: Open Rheumatology Journal - February 2, 2017 Category: Rheumatology Tags: Open Rheumatol J Source Type: research

Direct suppression of autoaggressive CD8+ T cells with CD80/86 blockade in CD8+ T cell-mediated polymyositis models of mice.
CONCLUSIONS: The CD80/86 blockade was effective in PM models of mice. Amelioration of CPIM indicates direct suppression of CD8+ T cells by the CD80/86 blockade. CTLA4-Ig should be a potential therapeutic agent of PM and other CD8+T cell-mediated diseases by suppressing both autoantigen-specific CD4+ and CD8+ T cells. PMID: 28134083 [PubMed - as supplied by publisher] (Source: Clinical and Experimental Rheumatology)
Source: Clinical and Experimental Rheumatology - February 1, 2017 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research

Myositis-specific autoantibodies and their association with malignancy in Italian patients with polymyositis and dermatomyositis
This study aims to characterize myositis-specific antibodies in a well-defined cohort of patients with idiopathic inflammatory myopathy and to determine their association with cancer. Sera from 40 patients with polymyositis, dermatomyositis, and controls were tested by protein and RNA immunoprecipitation to detect autoantibodies, and immunoprecipitation-Western blot was used for anti-MJ/NXP-2, anti-MDA5, and anti-TIF1 γ/α identification. Medical records were re-evaluated with specific focus on cancer. Anti-MJ/NXP-2 and anti-TIF1γ/α were the most common antibodies in dermatomyositis. In six dermatomyositis cases, we fou...
Source: Clinical Rheumatology - January 31, 2017 Category: Rheumatology Source Type: research

Sarcoplasmic MxA expression: A valuable marker of dermatomyositis
Conclusions: Sarcoplasmic MxA expression detected by immunohistochemistry is a more sensitive marker of DM than the conventional hallmarks, indicating its practical utility in the diagnosis of DM. It may well be included in the routine immunohistochemistry panel for myositis. Classification of evidence: This study provides Class II evidence that immunohistochemistry-detected sarcoplasmic MxA expression accurately identifies patients with dermatomyositis. (Source: Neurology)
Source: Neurology - January 29, 2017 Category: Neurology Authors: Uruha, A., Nishikawa, A., Tsuburaya, R. S., Hamanaka, K., Kuwana, M., Watanabe, Y., Suzuki, S., Suzuki, N., Nishino, I. Tags: Autoimmune diseases, Muscle disease ARTICLE Source Type: research

Hypokalemic myopathy in primary aldosteronism: A case report.
Authors: Wu C, Xin J, Xin M, Zou H, Jing L, Zhu C, Lei W Abstract Primary aldosteronism (PA) is a rare disorder. The majority of patients with PA present with typical features and are easily diagnosed. This disorder is usually diagnosed with hypokalemia, hypertension or an adrenal mass. However, patients with atypical symptoms may present a challenge for diagnosis and treatment. In the present study, a case of PA is described that presented with hypokalemic myopathy simulating polymyositis. The patient was a 44-year-old woman who presented with weakness and difficulty walking. The patient was initially suspected to...
Source: Experimental and Therapeutic Medicine - January 20, 2017 Category: Journals (General) Tags: Exp Ther Med Source Type: research

Use of intravenous immunoglobulin therapy for myositis: an audit in South Australian patients
In South Australia, between 2000 and 2014, 57 patients with idiopathic inflammatory myositis (IIM) were treated with intravenous immunoglobulin (IVIg). We reviewed disease characteristics to determine predictors of response to therapy and IVIg dosing and duration to identify opportunities to rationalise IVIg use. Patients with dermatomyositis/polymyositis had a response rate of 77% and were more likely than inclusion body myositis to respond to therapy. Consideration should be given to the use of the lowest possible dose of IVIg and to the undertaking of trials of cessation of IVIg in patients with stable IIM. (Source: Int...
Source: Internal Medicine Journal - January 10, 2017 Category: Internal Medicine Authors: Caroline Foreman, Paul Russo, Noelene Davies, Pravin Hissaria, Susanna Proudman, Tiffany Hughes, Vidya Limaye Tags: Brief Communications Source Type: research

Atorvastatin-induced necrotizing autoimmune myositis: An emerging dominant entity in patients with autoimmune myositis presenting with a pure polymyositis phenotype
Abstract: The general aim of this study was to evaluate the disease spectrum in patients presenting with a pure polymyositis (pPM) phenotype. Specific objectives were to characterize clinical features, autoantibodies (aAbs), and membrane attack complex (MAC) in muscle biopsies of patients with treatment-responsive, statin-exposed necrotizing autoimmune myositis (NAM). Patients from the Centre hospitalier de l’Université de Montréal autoimmune myositis (AIM) Cohort with a pPM phenotype, response to immunosuppression, and follow-up ≥3 years were included. Of 17 consecutive patients with pPM, 14 patients had a NAM, of w...
Source: Medicine - January 1, 2017 Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research

Serum level of DNase1l3 in patients with dermatomyositis/polymyositis, systemic lupus erythematosus and rheumatoid arthritis, and its association with disease activity
AbstractDNase1l3 is an endonuclease to degrade the chromatin of apoptotic or necrotic cells. Serum DNase1l3 may fulfill the function of clearance of chromatin released into the circulation by dying cells, which can trigger autoimmune responses. To date, it remains unclear whether serum DNase1l3 level associates with the pathogenesis of autoimmune diseases. Sixty-eight patients with dermatomyositis/polymyositis (DM/PM,n = 30), systemic lupus erythematosus (SLE,n = 20) and rheumatoid arthritis (RA,n = 18), as well as 26 healthy blood donors were enrolled in the present study. Serum levels of DNase1l3 were quantified by...
Source: Clinical and Experimental Medicine - December 29, 2016 Category: Research Source Type: research

Circulating CD8+CD28null T Cytotoxic Cells in Polymyositis —A Possible Biomarker? Comment on the Article by Pandya et al
(Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - December 27, 2016 Category: Rheumatology Authors: Florenzo Iannone, Giuseppe Lopalco, Alberto Cauli, Luca Cantarini Tags: Letters Source Type: research

Rituximab in the treatment of inflammatory myopathies: a review
Several uncontrolled studies have encouraged the use of rituximab (RTX) in patients with myositis. Unfortunately, the first placebo-phase trial to assess the efficacy of RTX in refractory myositis did not show a significant difference between the two treatment groups, and doubts have been expressed about its study design. In this review we present an up-to-date overview of the reported experiences of RTX therapy in myositis. A PubMed search was performed to find all the available cases of refractory myositis patients treated with RTX up to July 2015. The following terms were assessed: inflammatory myopathies OR anti-synthe...
Source: Rheumatology - December 26, 2016 Category: Rheumatology Authors: Fasano, S., Gordon, P., Hajji, R., Loyo, E., Isenberg, D. A. Tags: Myositis and Muscle Disease REVIEWS Source Type: research

The host defense peptide LL-37 a possible inducer of the type I interferon system in patients with polymyositis and dermatomyositis.
In conclusion, the present study supports our hypothesis that LL-37 may activate type I IFNs, which could initiate and perpetuate an inflammatory process. The prolonged exposure of the immune system to type I IFNs may eventually break tolerance and lead to autoimmune myositis. PMID: 28012697 [PubMed - as supplied by publisher] (Source: Journal of Autoimmunity)
Source: Journal of Autoimmunity - December 20, 2016 Category: Allergy & Immunology Authors: Lu X, Tang Q, Lindh M, Dastmlchi M, Alexanderson H, Popovic Silwerfeldt K, Agerberth B, Lundberg IE, Wick C Tags: J Autoimmun Source Type: research