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Inflammatory Myopathies

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https://www.readbyqxmd.com/read/27919547/granuloma-formation-in-a-patient-with-gne-myopathy-a-case-report
#1
Keiko Nakamura, Tsuyoshi Hamaguchi, Kenji Sakai, Daisuke Noto, Kenjiro Ono, Yukiko Hayashi, Ichizo Nishino, Masahito Yamada
We report a patient with GNE myopathy with a homozygous mutation (c.1505-4G>A) in GNE gene. The patient recognized progressive weakness of extremities at age 60. Neurological examination at age 65 revealed severe weakness and atrophy in the tibialis anterior muscles and distal predominant moderate weakness in the extremities. Muscle biopsy performed at age 65 showed myopathic changes with rimmed vacuoles, and the noteworthy finding was non-caseating epithelioid cell granuloma formation surrounded by numerous inflammatory cells...
November 18, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27917636/-immune-mediated-necrotizing-myopathy-associated-with-statin-treatment
#2
Jiří Vencovský
Immune mediated necrotizing myopathy (IMNM) is an acquired inflammatory myopathy. One form of the disease is associated with serum anti-HMGCR autoantibodies and significant proportion of patients has a history of statin treatment. The disease is rare with the incidence estimated around 23 cases/100 000 statin treated individuals. In contrast to direct statin toxicity, anti-HMGCR associated IMNM is an induced autoimmune disease.Patients suffer particularly from severe muscle weakness, which may be occasionally totally immobilizing...
2016: Casopís Lékar̆ů C̆eských
https://www.readbyqxmd.com/read/27916754/-autoantibodies-of-inflammatory-myopathies-update
#3
Shigeaki Suzuki
Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve the skeletal muscle as well as many other organs. In addition to a histological diagnosis at muscle biopsy, the clinical phenotypes of inflammatory myopathies can be defined by the presence of various autoantibodies that are originally detected by RNA or protein immunoprecipitation. However, the correlation between histological features and autoantibodies has not been fully elucidated. Immune-mediated necrotizing myopathy (IMNM), which is characterized by significant necrotic and regeneration muscle fibers with minimal or no inflammatory cell infiltration, is associated with the presence of autoantibodies...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27916753/-diagnosis-of-idiopathic-inflammatory-myopathy-a-muscle-pathology-perspective
#4
Michio Inoue, Ichizo Nishino
Idiopathic inflammatory myopathies are historically classified into polymyositis and dermatomyositis based on the presence or absence of skin lesions. Recently, however, a more histology-oriented classification into 6 subtypes has been proposed. The subtypes include dermatomyositis, polymyositis, inclusion body myositis, immune-mediated necrotizing myopathy, anti-synthetase syndrome, and non-specific myositis. With strict criteria applied, polymyositis is now extremely rare, while immune-mediated necrotizing myopathy is the most common among all inflammatory myopathies and is often associated with autoantibodies including those for signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR)...
December 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27912993/rehabilitation-in-chronic-graft-versus-host-disease
#5
REVIEW
Sean Robinson Smith, Arash Asher
Chronic graft-versus-host disease is a potentially debilitating complication of allogeneic hematopoietic stem cell transplantation. Due to the direct inflammatory effects of the disease on tissue, and the impact on muscle and bone of the high-dose glucocorticoid immunosuppression used to treat the disease, patients are at risk of developing multifactorial functional impairment. This review outlines the clinical assessment and rehabilitation interventions to manage aspects of the disease that cause the most impairment: involvement of the skin/fascial and cardiopulmonary organ systems, as well as steroid-induced myopathy and bone and joint destruction...
February 2017: Physical Medicine and Rehabilitation Clinics of North America
https://www.readbyqxmd.com/read/27908534/critical-review-of-the-role-of-intravenous-immunoglobulins-in-idiopathic-inflammatory-myopathies
#6
REVIEW
Sabrina Anh-Tu Hoa, Marie Hudson
OBJECTIVE: The aim of this review was to summarize key findings from the literature concerning the therapeutic role of intravenous immunoglobulins (IVIg) in idiopathic inflammatory myopathies (IIM), dissecting the evidence according to disease subtype and treatment indication, and to review the evidence relating to the mechanism of action of IVIg in IIM to ascertain rationale for continued research. METHODS: Medline (Ovid) and Pubmed databases were searched from inception to July 2016 using relevant keywords...
July 29, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/27897416/curcumin-an-effective-adjunct-in-patients-with-statin-associated-muscle-symptoms
#7
REVIEW
Amirhossein Sahebkar, Nikou Saboni, Matteo Pirro, Maciej Banach
In spite of the unequivocal efficacy of statins in reducing primary and secondary cardiovascular events, the use of these drugs in a considerable number of patients is limited because of statin intolerance, mainly statin-associated muscle symptoms (SAMS). SAMS encompass a broad spectrum of clinical presentations, including mild muscular aching and other types of myalgias, myopathy with the significant elevation of creatine kinase, and the rare but life-threatening rhabdomyolysis. Among several pathophysiologic mechanisms of SAMS, mitochondrial dysfunction is thought to be one of the main one...
September 22, 2016: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/27896437/-inflammatory-myopathies
#8
Britta Maurer
Inflammatory myopathies comprise heterogeneous, often multisystemic autoimmune diseases with muscle involvement as a common feature. The prognosis largely depends on a timely diagnosis and initiation of therapy. Given the complexity of these rare diseases, when an inflammatory myopathy is suspected patients should be referred to an expert center with established algorithms for the diagnostic work-up. The differential diagnostic exclusion of myositis mimics should ideally be carried out in close collaboration with neurologists and neuropathologists...
November 28, 2016: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/27894310/monitoring-change-in-volume-of-calcifications-in-juvenile-idiopathic-inflammatory-myopathy-a-pilot-study-using-low-dose-computed-tomography
#9
Maria Ibarra, Cynthia Rigsby, Gabrielle A Morgan, Christina L Sammet, Chiang-Ching Huang, Dong Xu, Ira N Targoff, Lauren M Pachman
BACKGROUND: Dystrophic calcifications may occur in patients with J uvenile Idiopathic Inflammatory Myopathy (JIIM) as well as other connective tissue and metabolic diseases, but a reliable method of measuring the volume of these calcifications has not been established. The purpose of this study is to determine the feasibility of low dose, limited slice, Computed Tomography (CT) to measure objectively in-situ calcification volumes in patients with JIIM over time. METHODS: Ten JIIM patients (eight JDM, two Overlap) with calcifications were prospectively recruited over a 2-year period to undergo two limited, low dose, four-slice CT scans...
November 29, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27890880/myopericarditis-in-a-case-of-anti-signal-recognition-particle-anti-srp-antibody-positive-myopathy
#10
Mariko Tanaka, Naoki Gamou, Hirohiko Shizukawa, Emiko Tsuda, Shun Shimohama
A 79 year-old female was admitted to our hospital because of high serum creatine kinase level together with proximal muscle weakness and pain on grasping. MRI revealed inflammatory changes in femoral muscles on both sides. Muscle biopsy showed size irregularity of muscle cells, and necrosis and regeneration of fibers. Study of antibodies was also consistent with the diagnostic criteria of anti-signal recognition particle (anti-SRP) antibody-positive myopathy. On admission, the patient required pericardiocentesis for the management of exudative pericarditis...
November 25, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27882118/atypical-miyoshi-distal-myopathy-a-case-report
#11
Meiling Wang, Yujie Guo, Yong Fu, Rui Jia, Gang Chen
Five distinct predominant distal myopathies have been identified with discrete clinical and genetic patterns. Miyoshi myopathy (MM; early adult-onset, type 2) is a subtype of dysferlinopathy. Furthermore, MM is the most common form of autosomal recessive distal myopathy. MM is typically characterized by muscular weakness, initially affecting the gastrocnemius or soleus muscle from the late teens or early adulthood. The present study reports a case of MM that was confirmed by pathological and immunohistochemical methods, in addition to a review of the relevant literature...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27878344/comparison-of-soluble-urokinase-plasminogen-activator-receptor-soluble-triggering-receptor-expressed-on-myeloid-cells-1-procalcitonin-and-c-reactive-protein-in-distinguishing-concurrent-bacterial-infection-from-idiopathic-inflammatory-myopathy
#12
Yizhi Xiao, Hui Luo, Bin Zhou, Xiaodan Dai, Jing Huang, Liping Duan, Yunhui You, Weiru Zhang, Hongjun Zhao, Yanli Xie, Yaou Zhou, Wangbin Ning, Tong Li, Sijia Liu, Honglin Zhu, Xiaoyun Xie, Ying Jiang, Shiyao Wu, Weijia He, Yisha Li
The aim of the study was to measure the diagnostic values of biomarkers of bacterial infection in idiopathic inflammatory myopathy (IIM) patients. The serum and clinical data of 82 IIM patients with/without bacterial infection were collected. Concentrations of soluble urokinase plasminogen activator receptor (suPAR), soluble triggering receptor expressed on myeloid cells 1 (sTREM-1), procalcitonin (PCT) and C-reactive protein (CRP) were measured in IIM patients and healthy controls. There were no significant differences in serum suPAR and sTREM-1 levels between healthy controls and non-infection IIM patients...
November 22, 2016: Rheumatology International
https://www.readbyqxmd.com/read/27876175/-anti-hmg-coa-reductase-necrotizing-myopathy-a-report-of-4-cases
#13
J Marti, I Kolev, A Lorleac'h, S Besnard, M Pihan, P Marcorelles
INTRODUCTION: Anti-3-hydroxy-3-méthylglutaryl-coenzyme A reductase antibody-associated necrotizing autoimmune myopathy has been recently described (2011). This myopathy is distinct from statin toxic myopathy. Our objective is to report on the clinical and para-clinical characteristics of this myopathy and to show the difficulties of therapeutic care. CASE REPORTS: We describe 4 cases of patients followed-up in Brittany, France. All data have been analyzed retrospectively...
November 18, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27865559/analysis-of-sexual-function-of-patients-with-dermatomyositis-and-polymyositis-through-self-administered-questionnaires-a-cross-sectional-study
#14
Fernando Henrique Carlos de Souza, Daniel Brito de Araújo, Clovis Artur Silva, Renata Miossi, Carmita Helena Najjar Abdo, Eloisa Bonfá, Samuel Katsuyuki Shinjo
INTRODUCTION: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM). OBJECTIVE: To assess sexual function in female patients with DM/PM. PATIENTS AND METHODS: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group...
November 1, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27861229/what-s-in-the-literature
#15
Nicholas J Silvestri, Gil I Wolfe, David Lacomis
In this edition, we focus on neuromuscular junction disorders and myopathy. The newly published international consensus guidelines for the management of myasthenia gravis are reviewed. In addition, various emerging treatment options for myasthenia, including the use of methotrexate, rituximab, subcutaneous immunoglobulin, and thymectomy, are discussed. Recent studies examining the clinical and genetic features of several forms of congenital myasthenia gravis are also highlighted. The clinical features and treatment of late-onset Pompe disease are reviewed, as are studies in facioscapulohumeral dystrophy, idiopathic inflammatory myopathies, and calpainopathy...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27861218/inclusion-body-myositis-what-most-impacts-patients-lives
#16
Cynthia Gibson, Nicholas E Johnson, Eileen Eastwood, Chad Heatwole
OBJECTIVE: Inclusion body myositis (IBM) is the most common form of idiopathic inflammatory myopathy in adults older than 50 years. Few studies have focused on the functional, physical, and social limitations of this disease. This study identifies pertinent symptoms that impact the health and daily function of patients with IBM. METHODS: We used semistructured interviews with 10 biopsy-confirmed adults with IBM to identify the psychological, physical, and functional limitations that have the greatest impact on the lives of patients with IBM...
December 2016: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/27859369/childhood-macrophagic-myofasciitis-a-series-from-the-indian-subcontinent
#17
Aanchal Kakkar, Madhu Rajeshwari, Aasma Nalwa, Vaishali Suri, Chitra Sarkar, Biswaroop Chakrabarty, Sheffali Gulati, Mehar C Sharma
INTRODUCTION: Macrophagic myofasciitis (MMF) is a rare disorder, reported mainly in European adults, with occasional childhood cases. We report a series of 6 patients with pediatric MMF from the Indian subcontinent. METHODS: Clinical details, creatine kinase levels, and results of electromyography are described for patients diagnosed with MMF. Fresh-frozen and formalin-fixed muscle biopsies were evaluated by hematoxylin-eosin staining, histochemistry, immunohistochemistry, and electron microscopy...
November 11, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27858925/-dermatomyositis-as-the-first-manifestation-of-small-cell-carcinoma-case-report-and-literature-review
#18
Herman Ahumada Piña, Luis Fuentes Cabrera, Rodrigo Selamé Glena, Daniel García Oneto, Andrea Jiménez Aguilar, Claudia Durán Herrera, Jorge Ávila Pérez, Maximiliano Cortés Latorre
Dermatomyositis is an autoimmune inflammatory myopathy associated with dermatologic findings. Its association with neoplasms is widely reported and has been shown to be higher in lung cancer, particularly small-cell carcinoma, probably as it originates from neuroendocrine cell precursors, as well as breast and ovarian cancer. The low frequency of dermatomyositis makes it difficult to suspect the underlying lesions among the diagnostic possibilities of systemic diseases with cutaneous manifestations. Therefore, the high probability of a neoplasm should always be considered, with reports of up to 75% in the eastern population and about 40% in general...
November 15, 2016: Medwave
https://www.readbyqxmd.com/read/27858897/anti-hmgcr-antibodies-in-systemic-sclerosis
#19
Marie Hudson, Yael Luck, Mathew Stephenson, May Y Choi, Mianbo Wang, Murray Baron, Marvin J Fritzler
The objective of this study was to investigate the frequency of autoantibodies to hydroxymethylglutaryl coenzyme A reductase (HMGCR) in systemic sclerosis (SSc) and associations with inflammatory myositis and statin exposure.This was a cross-sectional, multicenter study of 306 subjects from the Canadian Scleroderma Research Group cohort who had complete data on statin exposure and serology for anti-HMGCR antibodies assayed by an addressable laser bead immunoassay (ALBIA). Descriptive statistics were used to summarize the baseline characteristics of the patients and to compare subjects with and without anti-HMGCR antibodies...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27858337/development-and-evaluation-of-a-standardized-elisa-for-the-determination-of-autoantibodies-against-cn-1a-mup44-nt5c1a-in-sporadic-inclusion-body-myositis
#20
Sabine L Kramp, Dmitry Karayev, Guo Shen, Allan L Metzger, Robert I Morris, Eugene Karayev, Yvonne Lam, Richard M Kazdan, Ger J M Pruijn, Sandra Saschenbrecker, Cornelia Dähnrich, Wolfgang Schlumberger
PURPOSE: Sporadic inclusion body myositis (sIBM) is an autoimmune degenerative disease of the muscle, with inflammatory infiltrates and inclusion vacuoles. Its pathogenesis is not fully understood and the diagnosis is hampered by its imprecise characteristics, at times indistinguishable from other idiopathic inflammatory myopathies such as polymyositis and dermatomyositis. The diagnosis may be assisted by the detection of autoantibodies targeting Mup44, a skeletal muscle antigen identified as cytosolic 5'-nucleotidase 1A (cN-1A, NT5C1A)...
December 2016: Auto- Immunity Highlights
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