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Ya-Mei Zhang, Han-Bo Yang, Jing-Li Shi, He Chen, Xiao-Ming Shu, Xin Lu, Guo-Chun Wang, Qing-Lin Peng
Autoantibodies against poly-U-binding factor 60 kDa protein (PUF60) have been reported in Caucasian dermatomyositis (DM) patients. However, their clinical significance in idiopathic inflammatory myopathy (IIM) remains to be fully clarified. Our objective was to analyze the prevalence and clinical significance of anti-PUF60 antibodies in a large cohort of Chinese IIM patients. In our study, 388 IIM patients, 301 disease controls, and 167 healthy controls (HCs) were involved. An enzyme-linked immunosorbent assay (ELISA) was developed to detect serum anti-PUF60 levels and was validated using immunoblotting methods...
March 15, 2018: Clinical Rheumatology
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
Ying Hou, Yue-Bei Luo, Tingjun Dai, Kai Shao, Wei Li, Yuying Zhao, Jian-Qiang Lu, Chuanzhu Yan
The European Neuromuscular Centre (ENMC) pathological classification criteria of idiopathic inflammatory myopathies (IIMs) are debatable. The aim of this study was to explore their practicability and reproducibility. We conducted a retrospective analysis of 57 cases of IIMs excluding dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) by in-depth analysis of muscle biopsies and comparisons of the clinical characteristics among polymyositis (PM), non-specific myositis (NSM) and necrotizing autoimmune myopathy (NAM)...
March 7, 2018: Journal of Neuropathology and Experimental Neurology
Latika Gupta, Smriti Chaurasia, Puja Srivastava, Sanjay Dwivedi, Able Lawrence, Ramnath Misra
We studied the serum levels of B cell survival factors BAFF and APRIL in patients with idiopathic inflammatory myositis (IIM) and their relation with clinical and autoantibodies. Seventy-five patients (51 females and 24 males) with IIM (Bohan and Peter's criteria 1975) and 25 healthy adults were analyzed for BAFF, APRIL and IL-17 by ELISA, and myositis-specific and associated antibodies (MSA and MAA) using line immunoblot assay. Of the 75 patients, 59 were adults, 42 had Dermatomyositis (DM), and 17 had Polymyositis...
March 7, 2018: Clinical Rheumatology
Sandrine Herbelet, Elly De Vlieghere, Amanda Gonçalves, Boel De Paepe, Karsten Schmidt, Eline Nys, Laurens Weynants, Joachim Weis, Gert Van Peer, Jo Vandesompele, Jens Schmidt, Olivier De Wever, Jan L De Bleecker
Aims: Regeneration in skeletal muscle relies on regulated myoblast migration and differentiation, in which the transcription factor nuclear factor of activated T-cells 5 (NFAT5) participates. Impaired muscle regeneration and chronic inflammation are prevalent in myositis. Little is known about the impact of inflammation on NFAT5 localization and expression in this group of diseases. The goal of this study was to investigate NFAT5 physiology in unaffected myoblasts exposed to cytokine or hyperosmolar stress and in myositis...
2018: Frontiers in Physiology
Rita de Sousa Gameiro, Ana Isabel Alves Reis, Ana Cristina Grilo, Carla Noronha
Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis...
March 5, 2018: BMJ Case Reports
Chanyuan Wu, Qian Wang, Linrong He, Enhao Yang, Xiaofeng Zeng
BACKGROUND: Polymyositis and dermatomyositis (PM/DM) are systemic autoimmune diseases with multiple organ involvements that manifest as muscular and cutaneous disorders, interstitial lung disease (ILD) and malignancies. However, information concerning the outcomes and associated factors for PM/DM patients who are hospitalized is limited. METHODS: We retrospectively reviewed the medical charts of PM/DM patients admitted to a Chinese tertiary referral hospital (Peking Union Medical College Hospital, PUMCH) from 2008 to 2014...
2018: PloS One
David Lacomis
This review is focused on recent reports of sporadic inclusion body myositis (sIBM), myopathy in patients with human immunodeficiency virus type 1 (HIV) infection, and necrotizing autoimmune myopathy with antibodies to signal recognition particle. The sIBM articles cover associations with certain genetic polymorphisms, the possible pathogenic role of anti-cytosolic 5'-nucleotidase 1A antibody, and disease-related burden and health care costs. Another article addressed the possible pathogenic role of signal recognition particle antibody in necrotizing myopathy...
March 2018: Journal of Clinical Neuromuscular Disease
Or Carmi, Maya Berla, Evgeny Edelstein, Yair Levy
No abstract text is available yet for this article.
February 14, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Limesh Marisiddappa, Atul M Desai, Prashanth G Kedlaya, Renuka Sathish
Polymyositis (PM) is a rare heterogeneous group of disorders with frequent multisystem involvement including uncommon renal manifestations. Acute kidney injury (AKI) as the primary manifestation of PM is extremely rare. Herein, we report a case of recurrent episodic AKI in an adult female who was subsequently diagnosed to have PM.
January 2018: Saudi Journal of Kidney Diseases and Transplantation
Pratyusha Bikkina, Swapna Kotha, Zakir Ali
Idiopathic inflammatory myositis is characterized by rapidly progressive, symmetric weakness of the muscles that produce severe disability. In the majority of these patients, myositis appears to be a paraneoplastic feature associated with cancer. Fluorodeoxyglucose positron emission tomography-computed tomography has been increasingly used in the detection and evaluation of occult malignancy responsible for the paraneoplastic syndromes.
January 2018: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
O Pelton, G Chatte
Acute or subacute interstitial lung diseases from autoimmune origins are especially hard to diagnose but have to be detected promptly. We illustrate this necessity with three case reports. One case of paraneoplasic polymyositis, one case of interstitial lung disease caused by a connectivite and one case of interstitial lung disease related to an anti-synthetase syndrome. The subject is to alert the practitioners to the early search of extra pulmonary signs, autoantibodies analysis in the objective to set up quickly the right treatment...
February 6, 2018: Revue de Pneumologie Clinique
Helene Alexanderson, Malin Regardt, Christina Ottosson, Li Alemo Munters, Maryam Dastmalchi, Lara Dani, Ingrid E Lundberg
OBJECTIVE: To investigate muscle impairment (isometric and dynamic) and disease activity during the first year after diagnosis of polymyositis (PM) and dermatomyositis (DM), and to study the relationship between muscle impairment, patient-reported health, and disease activity. METHODS: Seventy-two patients enrolled in the Swedish Myositis Register, 2003-2010, were followed prospectively. The Manual Muscle test (MMT-8; isometric muscle strength), the Functional Index of myositis test (FI-2; dynamic, repetitive muscle function), and disease activity (6-item core set) were retrieved at the time of diagnosis, and after 6 and 12 months...
February 1, 2018: Journal of Rheumatology
Yanqing Li, Peihong Wang, Lei Li, Fei Wang, Yuxiu Liu
Objective: Polymyositis and dermatomyositis (PM/DM) have been implicated in the development of venous thromboembolism (VTE). Previous studies investigating the association between PM/DM and VTE risk had yielded inconsistent findings. The aim of this study was to precisely estimate this association by meta-analysis of all available publications. Methods: Two investigators independently performed a comprehensive literature search in databases of PubMed, Embase, and the Cochrane Library for eligible studies...
2018: Therapeutics and Clinical Risk Management
Airi Nishimi, Takeo Isozaki, Shinichiro Nishimi, Sho Ishii, Takahiro Tokunaga, Hidekazu Furuya, Kuninobu Wakabayashi, Tsuyoshi Kasama
The "A disintegrin and metalloprotease" (ADAM) family is thought to play an important role in tissue destruction and inflammatory reactions. ADAM-17 was first described as the protease responsible for tumor necrosis factor (TNF)-α shedding. Here, we have shown the expression of ADAM-17 in inflammatory myopathy and demonstrated the role of inflammation in interstitial lung diseases (ILD). ADAM-17 in inflammatory myopathy serum [polymyositis (n = 26), dermatomyositis (n = 34), and clinically amyopathic dermatomyositis (n = 10)] and healthy control (n = 19) was measured using enzyme-linked immunosorbent assay...
February 6, 2018: Clinical Rheumatology
Shiro Matsubara, Kota Bokuda, Yuri Asano, Ryo Morishima, Keizo Sugaya, Kazuhito Miyamoto, Reiji Koide, Takashi Komori, Shigeaki Suzuki, Ichizo Nishino
Immune-mediated necrotizing myopathy (IMNM) associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) autoantibodies occurs in patients both with and without history of statin-intake. The mechanisms of muscle fiber degeneration in this condition remain unknown. We studied pathological changes in muscle biopsies from three patients lacking history of statin-intake. Ultrastructural observations showed accumulation of degenerating mitochondria, glycogen granules and autophagic vacuoles, forming large composites in three cases, along with various nonspecific changes...
January 11, 2018: Neuromuscular Disorders: NMD
Enrique Melguizo Madrid, Patricia Fernández Riejos, Francisco Javier Toyos Sáenz de Miera, Berta Fernández Pérez, Concepción González Rodríguez
Idiopathic inflammatory myopathies are a heterogeneous group of potentially treatable myopathies. They are classified, on the basis of clinical and histopathological features, into four subtypes: dermatomyositis, polymyositis, necrotizing autoimmune myositis and inclusion-body myositis. Myositis-associated antibodies and myositis-specific autoantibodies are frequently found in patients with idiopathic inflammatory myopathies, and are useful in the diagnosis and classification. Anti-histidyl transfer RNA synthetase antibody is the most widely prevalent and is highly specific for polymyositis...
January 25, 2018: Reumatología Clinica
Małgorzata Peregud-Pogorzelska, Małgorzata Zielska, Jarosław Kaźmierczak
Polymyositis (PM) is an autoimmune disease characterized by the involvement of multiple internal organs, including the cardiovascular system. The involvement of heart is observed in up to 75% of patients with PM. Conduction and rhythm disorders are one of the most common cardiological abnormalities in these patients. The presented clinical case is the patient where ventricular arrhythmia (in the form of multiple premature ventricular extrasystoles) was the first symptom of polymyositis.
January 30, 2018: Annals of Noninvasive Electrocardiology
Ho So, Ricky W-K Ip, Victor T-L Wong, Ronald M-L Yip
AIM: To compare the prevalence of the anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) in Hong Kong Chinese patients with dermatomyositis (DM) and polymyositis (PM); in addition, to examine the association of anti-MDA5 Ab and the clinical characteristics of these patients. METHODS: Twenty consecutive existing patients with DM being followed up at the Rheumatology Clinic of Kwong Wah Hospital, Hong Kong were recruited. Twenty patients with PM were recruited from the same clinic as the controls...
January 30, 2018: International Journal of Rheumatic Diseases
Syahrul Sazliyana Shaharir, Adawiyah Jamil, Sumitro Kosasih, Low Soo Fin, Radhika Sridharan, Suria Hayati Md Pauzi
A 59-year-old man presented with proximal myopathy, myalgia, and weight loss, with the initial markedly elevated serum creatine kinase at 11,000 U/L. Due to his refusal for muscle biopsy, he was initially treated as inflammatory myositis and responded well with the corticosteroids. However, he subsequently had a relapse of the symptoms with more extensive systemic involvement, i.e., hypercalcemia, lymphadenopathy and subcutaneous nodules. Finally, a biopsy of the thigh and subcutaneous nodule revealed non-caseating granulomatous inflammation, consistent with sarcoidosis...
December 2017: Acta Medica Iranica
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