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Animal model dermatomyositis

Hirokazu Sasaki, Hitoshi Kohsaka
No abstract text is available yet for this article.
September 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
Naoko Okiyama, Manabu Fujimoto
A set of histopathological elements, that is death of epidermal basal cell layer keratinocytes and inflammatory cell infiltration, distinguishes lichenoid tissue reaction (LTR)/interface dermatitis (IFD) from other inflammatory mucocutaneous diseases with histological findings of superficial perivascular dermatitis. The LTR/IFD is observed in inflammatory mucocutaneous diseases such as lichen planus, Stevens-Johnson syndrome/toxic epidermal necrolysis, acute graft-versus-host disease, lupus erythematosus and dermatomyositis...
June 2015: Journal of Dermatological Science
Guo Li, Li Yuzhen, Chen Yi, Chen Xiaoxiang, Zhou Wei, Zhu Changqing, Ye Shuang
BACKGROUND: Paraquat (PQ) poisoning is a lethal toxicological challenge that served as a disease model of acute lung injury and pulmonary fibrosis, but the mechanism is undetermined and no effective treatment has been discovered. METHODS AND FINDINGS: We demonstrated that PQ injures mitochondria and leads to mtDNA release. The mtDNA mediated PBMC recruitment and stimulated the alveolar epithelial cell production of TGF-β1 in vitro. The levels of mtDNA in circulation and bronchial alveolar lavage fluid (BALF) were elevated in a mouse of PQ-induced lung injury...
2015: BioMed Research International
Jan D Lünemann, Falk Nimmerjahn, Marinos C Dalakas
Intravenous immunoglobulin (IVIg)-a preparation of polyclonal serum IgG pooled from thousands of blood donors-has been used for nearly three decades, and is proving to be an efficient anti-inflammatory and immunomodulatory treatment for a growing number of neurological diseases. Evidence from controlled clinical trials has established IVIg as a first-line therapy for Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy. IVIg is also an effective rescue therapy in some patients with worsening myasthenia gravis, and is beneficial as a second-line therapy for dermatomyositis and stiff-person syndrome...
February 2015: Nature Reviews. Neurology
Li Zhang, Xiao-Hong Fu, Yong Yu, Ruo-Hong Shui, Chun Li, Hai-Ying Zeng, Yu-Lei Qiao, Li-Yan Ni, Qiang Wang
Cathepsin B (CB) is involved in the turnover of proteins and has various roles in maintaining the normal metabolism of cells. In our recent study, CB is increased in the muscles of polymyositis/dermatomyositis (PM/DM). However, the role of CB in interstitial lung disease (ILD) has not been reported. ILD is a frequent complication of PM/DM, which is the leading cause of death in PM/DM. It carries high morbidity and mortality in connective tissue diseases, characterized by an overproduction of inflammatory cytokines and induced fibrosis, resulting in respiratory failure...
January 2015: Laboratory Investigation; a Journal of Technical Methods and Pathology
Zhen-Rui Shi, Guo-Zhen Tan, Zhen Meng, Min Yu, Kai-Wen Li, Jing Yin, Kai-Hua Wei, Yi-Jin Luo, Shu-Qing Jia, Shu-Juan Zhang, Jian Wu, Xiang-Bin Mi, Liangchun Wang
OBJECTIVE: The specific autoantibodies and antigens that mediate systemic lupus erythematosus (SLE)-related organ injuries remain largely unknown. This study was undertaken to investigate the antibody-mediated immune response that leads to SLE skin lesions. METHODS: The study included 85 SLE patients with lupus-specific skin lesions and 31 without skin lesions. The reactivity of serum antibody with skin antigens was determined by immunoblotting using human foreskin as the substrate...
January 2015: Arthritis & Rheumatology
Payam Mohassel, Paul Rosen, Livia Casciola-Rosen, Katherine Pak, Andrew L Mammen
OBJECTIVE: Autoantibodies against transcription intermediary factor 1γ (TIF1γ) are found in many patients with dermatomyositis (DM). Although TIF1γ is known to play a role in the differentiation of other tissues, its functional role in muscle regeneration has not been elucidated. This study was undertaken to explore the regulation and functional role of this protein during muscle differentiation and regeneration. METHODS: TIF1γ expression was analyzed in human muscle biopsy specimens using immunofluorescence microscopy...
January 2015: Arthritis & Rheumatology
Robert W Georgantas, Katie Streicher, Steven A Greenberg, Lydia M Greenlees, Wei Zhu, Philip Z Brohawn, Brandon W Higgs, Meggan Czapiga, Christopher A Morehouse, Anthony Amato, Laura Richman, Bahija Jallal, Yihong Yao, Koustubh Ranade
OBJECTIVE: The molecular basis of inflammatory myopathies such as dermatomyositis (DM), polymyositis, and inclusion body myositis, which share the characteristics of chronic muscle inflammation and skeletal muscle wasting, are poorly understood. As such, effective targeted treatments for these diseases are lacking, resulting in critical unmet medical needs for these devastating diseases. The purpose of this study was to identify possible new targets for drug development by exploring the mechanism by which inflammation may play a role in the pathology of the inflammatory myopathies...
April 2014: Arthritis & Rheumatology
Masaya Yokota, Kotaro Suzuki, Koji Tokoyoda, Kazuyuki Meguro, Junichi Hosokawa, Shigeru Tanaka, Kei Ikeda, Takashi Mikata, Toshinori Nakayama, Hitoshi Kohsaka, Hiroshi Nakajima
INTRODUCTION: In addition to the pivotal roles of mast cells in allergic diseases, recent data suggest that mast cells play crucial roles in a variety of autoimmune responses. However, their roles in the pathogenesis of autoimmune skeletal muscle diseases have not been clarified despite their distribution in skeletal muscle. Therefore, the objective of this study is to determine the roles of mast cells in the development of autoimmune skeletal muscle diseases. METHODS: The number of mast cells in the affected muscle was examined in patients with dermatomyositis (DM) or polymyositis (PM)...
2014: Arthritis Research & Therapy
Shervin Assassi, Maureen D Mayes
PURPOSE OF REVIEW: The purpose of this study is to review recent hypothesis-driven studies that utilize global gene expression data for elucidating the molecular basis of systemic sclerosis (SSc) and its various clinical manifestations. RECENT FINDINGS: The longitudinal skin gene expression studies indicate that the previously identified molecular subsets are stable over time and might identify inherent subgroups of SSc patients. Skin transcript follow-up studies indicate that the Wnt/β-catenin pathway plays an important role in promotion of fibrogenesis in fibroblasts and preadipocytes...
November 2013: Current Opinion in Rheumatology
Hans H Goebel, Werner Stenzel
Electron microscopy is an essential component of myopathology, both in diagnostics and research of neuromuscular diseases. Although recently reduced in the diagnostic armamentarium, it has greatly been expanded to mouse models in research. Mostly it is descriptive, but a few additional techniques in combination with transmission electron microscopy have been employed. Foremost among them is immunoelectron microscopy, which assists in guiding molecular analysis in hereditary conditions, but may be vital in diagnostics of certain acquired entities, e...
October 2013: Ultrastructural Pathology
Sara Franzi, Mohammad Salajegheh, Remedios Nazareno, Steven A Greenberg
INTRODUCTION: Type 1 interferon (IFN)-inducible genes and their inducible products are upregulated in dermatomyositis muscle. Of these, IFN-stimulated gene 15 (ISG15) is one of the most upregulated, suggesting its possible involvement in the pathogenesis of this disease. To test this postulate, we developed a model of type 1 IFN mediated myotube toxicity and assessed whether or not downregulation of ISG15 expression prevents this toxicity. METHODS: Mouse myoblasts (C2C12 cell line) were cultured in the presence of type 1 or type 2 IFNs and ISG15 expression assessed by microarray analysis...
2013: PloS One
Eleonora Ballanti, Carlo Perricone, Elisabetta Greco, Marta Ballanti, Gioia Di Muzio, Maria Sole Chimenti, Roberto Perricone
The complement system is a component of the innate immune system. Its main function was initially believed to be limited to the recognition and elimination of pathogens through direct killing or stimulation of phagocytosis. However, in recent years, the immunoregulatory functions of the complement system were demonstrated and it was determined that the complement proteins play an important role in modulating adaptive immunity and in bridging innate and adaptive responses. When the delicate mechanisms that regulate this sophisticated enzymatic system are unbalanced, the complement system may cause damage, mediating tissue inflammation...
July 2013: Immunologic Research
Rawad Nasr, Ann M Reed, Erik J Peterson
PURPOSE OF REVIEW: Establishing diagnoses and distinguishing active disease from chronic injury remain significant clinical challenges in idiopathic inflammatory myopathies (IIM). Recent 'discovery' approaches utilizing novel genomic and proteomic techniques have revealed candidate molecular biomarkers to augment clinical and classical histological data. RECENT FINDINGS: Whole blood and serum Type 1 interferons (IFN-1) and IFN-1 inducible genes are gaining traction as disease biomarkers in IIM...
November 2012: Current Opinion in Rheumatology
William Coley, Sree Rayavarapu, Gouri S Pandey, Richard L Sabina, Jack H Van der Meulen, Beryl Ampong, Robert L Wortmann, Rashmi Rawat, Kanneboyina Nagaraju
OBJECTIVE: It is generally believed that muscle weakness in patients with polymyositis and dermatomyositis is due to autoimmune and inflammatory processes. However, it has been observed that there is a poor correlation between the suppression of inflammation and a recovery of muscle function in these patients. This study was undertaken to examine whether nonimmune mechanisms also contribute to muscle weakness. In particular, it has been suggested that an acquired deficiency of AMP deaminase 1 (AMPD1) may be responsible for muscle weakness in myositis...
November 2012: Arthritis and Rheumatism
Lucie Leveque, Kiarash Khosrotehrani
The origins of autoimmunity are still elusive despite significant advances in immunology. There is cumulative evidence that, beyond simple genetics, the maternal environment plays a critical role in the development of common autoimmune disorders, such as multiple sclerosis or diabetes. In recent years, the trafficking of maternal cells to the offspring has been clearly demonstrated. This microchimerism represents the very first immunological event in fetal life. The number of persisting maternal cells has been associated with several autoimmune disorders such as systemic sclerosis, juvenile dermatomyositis and diabetes...
July 2011: Chimerism
Mikako Ito, Tohru Ibi, Ko Sahashi, Masashi Ichihara, Masafumi Ito, Kinji Ohno
BACKGROUND: Molecular hydrogen has prominent effects on more than 30 animal models especially of oxidative stress-mediated diseases and inflammatory diseases. In addition, hydrogen effects on humans have been reported in diabetes mellitus type 2, hemodialysis, metabolic syndrome, radiotherapy for liver cancer, and brain stem infarction. Hydrogen effects are ascribed to specific radical-scavenging activities that eliminate hydroxyl radical and peroxynitrite, and also to signal-modulating activities, but the detailed molecular mechanisms still remain elusive...
2011: Medical Gas Research
Dawei Li, Yuji Wang, Nanwei Xu, Qianghua Wei, Min Wu, Xiaofeng Li, Ping Zheng, Sai Sun, Yuli Jin, Gailian Zhang, Ruomin Liao, Ping Zhang
INTRODUCTION: Follistatin-like protein 1 (FSTL1) is a proinflammation mediator implicated in arthritis in rodent animal models. The present study is aimed at assessing FSTL1 levels in systemic autoimmune diseases and correlating them with disease activity in patients with rheumatoid arthritis (RA). METHODS: Serum FSTL1 levels from 487 patients with systemic autoimmune diseases and 69 healthy individuals were measured by enzyme-linked immunosorbent assay (ELISA)...
2011: Arthritis Research & Therapy
Jean Sibilia, Emmanuel Chatelus, Alain Meyer, Jacques-Eric Gottenberg, Christelle Sordet, Joëlle Goetz
The inflammatory myopathies are a group of quite proteiform, systemic auto-immune diseases which include polymyositis, dermatomyositis and inclusion body myopathies. To facilitate the diagnosis, classification criteria (Bohan and Peter, 1975) have been proposed, based essentially on clinical criteria. In addition, over the past fifteen years, auto-antibodies characterizing certain forms of inflammatory myopathy have been identified. One distinguishes schematically: auto-antibodies specific for myositis and auto-antibodies sometimes associated with myositis...
October 2010: La Presse Médicale
Christina F Pelajo, Jorge M Lopez-Benitez, Laurie C Miller
Vitamin D levels depend on many variables, including sun exposure, age, ethnicity, body mass index, use of medications and supplements. A much higher oral vitamin D intake than the current guidelines is necessary to maintain adequate circulating 25(OH)D levels in the absence of UVB radiation of the skin. In addition to the traditional known metabolic activities, vitamin D has been shown to modulate the immune system, and its deficiency has been linked to the development of several autoimmune disorders including type 1 diabetes and multiple sclerosis...
May 2010: Autoimmunity Reviews
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