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J Dermatol 2018 Dec 18. Epub 2018 Dec 18.

Department of Internal Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.

Although rheumatologists, neurologists and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of the present study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor and Welfare, Japan. Read More

Rheumatol Int 2018 Dec 14. Epub 2018 Dec 14.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

Juvenile dermatomyositis (JDM) is the most common childhood idiopathic inflammatory myopathy (IIM). It is characterized by the classic skin rash in the form of Gottron papules and heliotrope rash, and symmetric proximal muscle weakness. Renal involvement in JDM is rare which includes acute kidney injury and glomerulonephritis. Read More

Int J Rheum Dis 2018 Dec 11. Epub 2018 Dec 11.

Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.

Objectives: To identify factors associated with deterioration of pulmonary function with disproportional decline in diffusing capacity for carbon monoxide (DLCO) relative to forced vital capacity (FVC) in patients with dermatomyositis (DM) and polymyositis (PM).

Methods: This retrospective cohort study included patients with DM and PM, in whom serial pulmonary function tests were available. Changes in FVC and DLCO over time were estimated using a linear mixed-effects model. Read More

Epigenomics 2019 Jan 7;11(1):23-33. Epub 2018 Dec 7.

Department of Rheumatology, Xiangya Hospital, Central South University, 87 Xiangya Road, Changsha, Hunan 410008, PR China.

Aim: Dermatomyositis (DM) and polymyositis (PM) are refractory systemic autoimmune diseases with unknown pathogenesis. miRNAs is an important epigenetic mechanism to regulate gene expression.

Methods: We performed whole miRNAs analysis, transcription analysis and the association between miRNAome and mRNAome. Read More

J Allergy Clin Immunol 2018 Dec 7. Epub 2018 Dec 7.

Institut National de la Santé et de la Recherche Médicale; Centre de Recherche des Cordeliers, Equipe-Immunopathologie et Immunointervention Thérapeutique; Sorbonne Université, Paris, F-75006, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, F-75006, France. Electronic address:

Background: Therapeutic normal immunoglobulin G or intravenous immunoglobulin (IVIG) exerts anti-inflammatory effects via several mutually nonexclusive mechanisms. Recent data in mouse models of autoimmune diseases suggest that IVIG induces IL-4 in basophils by enhancing IL-33 in SIGN-R1 innate cells. However, translational insight on these data is lacking. Read More

Rheumatol Int 2018 Dec 5. Epub 2018 Dec 5.

Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.

Recent studies have shown a high prevalence of dyslipidemia in patients with systemic autoimmune myopathies (SAM). However, little is known about the safety of the use of statins in these patients, and this gap in research motivated the accomplishment of the present study. In a retrospective cohort study conducted from 2004 to 2018, 250 patients with SAM were evaluated, and 24 patients had stable forms of SAM (16 dermatomyositis, 1 polymyositis and 7 antisynthetase syndrome) but had dyslipidemia and had received statins. Read More

Mod Rheumatol 2018 Nov 28:1-20. Epub 2018 Nov 28.

a Institute of Rheumatology, Tokyo Women's Medical University , Tokyo , Japan.

Objectives: We aimed to evaluate the usefulness of serum KL-6 for interstitial lung disease (ILD) with polymyositis/dermatomyositis (PM/DM).

Methods: All consecutive and previously untreated adult patients with PM/DM who were admitted to our hospital from 2010 to 2015 were included. The associations between serum KL-6 levels and clinical information were retrospectively analyzed. Read More

Pediatr Rheumatol Online J 2018 Nov 20;16(1):72. Epub 2018 Nov 20.

Division of Rheumatology and Immunology, The Affiliated Children's Hospital, Capital Institute of Pediatrics, Beijing, 100020, China.

Objective: To follow up the refractory juvenile dermatomyositis (JDM) with autologous hematopoietic stem cell transplantation (AHSCT) in a long time and to investigate whether AHSCT is effective and safe to treat refractory JDM.

Methods: We collected the AHSCT and follow-up data of three patients with refractory JDM who received autologous peripheral blood CD34+ cell transplantation in our hospital between June 2004 and July 2015. Those data include: hight, weight, routine blood and urine tests, ESR, CK, ALT, AST, LDH, renal functional tests, lymphocyte subpopulations, HRCT and muscle MRI. Read More

Scand J Immunol 2018 Nov 19:e12732. Epub 2018 Nov 19.

Division of Rheumatology, Department of Medicine, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.

We aimed to evaluate in vivo effects of abatacept on phenotypes of T and B cells in the circulation of myositis patients in a sub-study of the ARTEMIS trial. Twelve patients with paired frozen PBMCs before and after 6-month abatacept treatment were included in this sub-study where mass cytometry (CyTOF) was chosen as a technology to be tested for its utility in a real-life clinical immune monitoring setting. Using CyTOF, the peripheral T cell phenotypes demonstrated considerable variation over time and between individuals precluding the identification of treatment-specific changes. Read More

Ocul Immunol Inflamm 2018 Nov 16:1-9. Epub 2018 Nov 16.

b Department of Ophthalmology, Faculty of Medicine , University of Debrecen , Debrecen , Hungary.

Purpose: To evaluate corneal parameters in patients with polymyositis (PM) and dermatomyositis (DM) and compare them with those of healthy controls.

Methods: A total of 43 PM and 32 DM patients and 93 controls were enrolled in this cross-sectional, observational, case-control study. Corneal parameters were evaluated by Pentacam. Read More

Pediatr Rheumatol Online J 2018 Nov 13;16(1):70. Epub 2018 Nov 13.

Division of Rheumatology, Departments of Pediatrics and Medical Social Sciences, Ann & Robert H. Lurie Children's Hospital of Chicago/Northwestern University Feinberg School of Medicine, 225 E Chicago Ave Box 50, Chicago, IL, 60611, USA.

Background: Juvenile dermatomyositis (JDM) is a rare autoimmune disease that causes significant morbidity and quality of life impairment. Little is known about the inpatient burden of JDM in the US. Our goal was to determine the prevalence and risk factors for hospitalization with juvenile dermatomyositis and assess inpatient burden of JDM. Read More

Med Sci (Paris) 2018 Nov 12;34 Hors série n°2:35-38. Epub 2018 Nov 12.

Inserm U955 Team 10, Paris Est-Créteil University, Créteil, France.

Dysimmune and inflammatory myopathies (DIMs) affect around 14/100,000 people worldwide. Based on immupour nopathological criteria, DIMs are divided in four groups: (1) polymyositis (PM)/inclusion body myositis (IBM), (2) dermatomyositis (DM), (3) immune-mediated necrotizing myopathies (IMNM) and (iv) overlapping myositis including anti-synthetase syndrome (ASS). ASS and PM/IBM are characterized by the activation of inflammation with lymphocytic infiltrations. Read More

Int J Rheum Dis 2018 Nov 5. Epub 2018 Nov 5.

Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.

Objective: Anti-transcriptional intermediary factor 1 (TIF1) antibody is associated with idiopathic inflammatory myopathies (IIMs). The aim of this study was to investigate the expression of TIF1s in IIMs.

Method: TIF1α, β or γ expression in the skin and muscle of patients and controls was studied by immunohistochemistry. Read More

Clin Rheumatol 2018 Nov 3. Epub 2018 Nov 3.

Rheumatology Department, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046, Madrid, Spain.

Objective: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD).

Patients And Methods: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Read More

Neuropathol Appl Neurobiol 2018 Oct 31. Epub 2018 Oct 31.

Developmental Biology of Birth Defects, Developmental Biology and Cancer Programme, UCL GOS Institute of Child Health, UCL London, UK.

Aim: Juvenile idiopathic inflammatory myopathies (IIM) have been recently reclassified into clinico-serological subgroups. Myopathological correlates of the subgroups are incompletely understood.

Methods: We studied muscle biopsies from 101 children with clinically and serologically-defined juvenile IIM from the UK JDM Cohort and Biomarker Study by applying the international JDM score tool, myopathological review, and C5b-9 complement analysis. Read More

Pan Afr Med J 2018;30:80. Epub 2018 May 29.

Department of Neurosurgery, Hospital Imaculada Conceição da Sociedade Portuguesa de Beneficência, Ribeirão Preto, São Paulo, Brazil.

Neurotherapeutics 2018 Oct;15(4):976-994

Department of Neurology, Brigham and Women's Hospital and Harvard Medical School, 60 Fenwood Road, Boston, MA, 02115, USA.

The major forms of autoimmune myopathies include dermatomyositis (DM), polymyositis (PM), myositis associated with antisynthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). While each of these conditions has unique clinical and histopathological features, they all share an immune-mediated component. These conditions can occur in isolation or can be associated with systemic malignancies or connective tissue disorders (overlap syndromes). Read More

Ther Adv Musculoskelet Dis 2018 Oct 7;10(10):201-207. Epub 2018 Oct 7.

Octapharma France, Boulogne-Billancourt, France.

Immunoglobulin (Ig) therapy is used to treat a wide range of immunodeficiencies and autoimmune diseases; While, its clinical benefit has been demonstrated in several studies, Ig therapy is associated with a risk of systemic adverse effects. As such, Onset of renal impairment, including acute renal failure, osmotic nephrosis and renal insufficiency, after immunoglobulin administration is rare, but is one of the most significant concerns related to intravenous Ig use at immunomodulatory doses. However, only few studies have investigated the safety of subcutaneous Ig (SCIg) in relation to these rare conditions. Read More

Eur J Radiol 2018 Oct 9;107:26-32. Epub 2018 Aug 9.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1, Tomioka-Higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address:

Objective: Little has been reported on the radiological and pathological findings of interstitial pneumonia in mixed connective tissue disease (MCTD). There may be possible difference in treatment response and prognosis between the imaging patterns of systemic sclerosis (SSc)-like and polymyositis/dermatomyositis (PM/DM)-like. The purpose of this study was to examine whether the radiological images of interstitial pneumonia in MCTD presented SSc-like or PM/DM-like pattern, and to assess whether the imaging patterns corresponded to clinical and pathological features. Read More

Dtsch Med Wochenschr 2018 Oct 4;143(20):1472-1476. Epub 2018 Oct 4.

Ehem. Ärztlicher Leiter der Rheumaklinik und des Rheumaforschungsinstituts Aachen.

Idiopathic inflammatory myopathies (IIM) are a rare and clinically polymorphic and heterogenous group of myositis syndromes. Myositis is part of a systemic autoimmune disorder with various extramuscular manifestations affecting skin, lungs, joints, esophagus and other organ systems. Most myositis patients have autoantibodies against non organspecific antigens. Read More

J Am Acad Orthop Surg 2018 Oct 2. Epub 2018 Oct 2.

From the Department of Orthopaedic Surgery, University of Alabama at Birmingham Hospital, Birmingham, AL.

Introduction: Orthopaedic surgeons are wary of patients with neuromuscular (NM) diseases as a result of perceived poor outcomes and lack of data regarding complication risks. We determined the prevalence of patients with NM disease undergoing total joint arthroplasty (TJA) and characterized its relationship with in-hospital complications, prolonged length of stay, and total charges.

Methods: Data from the Nationwide Inpatient Sample from 2005 to 2014 was used for this retrospective cohort study to identify 8,028,435 discharges with total joint arthroplasty. Read More

J Med Invest 2018 ;65(3.4):251-257

Department of Respiratory Medicine, Okinawa Chubu Hospital.

Background: Dermatomyositis (DM) and polymyositis (PM) often have association with interstitial lung disease (ILD) which have disease specific autoantibody.

Methodology: We reviewed medical records of DM/PM associated ILD from January 2000 to December 2017 according to the autoantibody.

Result: We identified 52 patients, of whom 30 were antibody negative, 18 had anti aminoacyl-tRNA synthetases (ARS) antibodies and 4 had anti melanoma differentiation-associated gene (MDA)-5 antibody. Read More

J Immunol 2018 Nov 1;201(9):2832-2841. Epub 2018 Oct 1.

Division of Life Sciences, College of Life Sciences and Biotechnology, Korea University, Seoul 02841, Republic of Korea;

In addition to essential roles in protein synthesis, lysyl-tRNA synthetase (KRS) is secreted to trigger a proinflammatory function that induces macrophage activation and TNF-α secretion. KRS has been associated with autoimmune diseases such as polymyositis and dermatomyositis. In this study, we investigated the immunomodulatory effects of KRS on bone marrow-derived dendritic cells (DCs) of C57BL/6 mice and subsequent polarization of Th cells and analyzed the underlying mechanisms. Read More

Pediatr Rev 2018 Oct;39(10):501-515

Division of Pediatric Rheumatology, Department of Pediatrics, University of Minnesota and the University of Minnesota Masonic Children's Hospital, Minneapolis, MN.

Am J Med Case Rep 2018 23;6(8):157-160. Epub 2018 Aug 23.

Divisions of Cardiovascular Disease and Rheumatology, Department of Internal Medicine, State University of New York, Downstate Medical Center, Brooklyn, New York, U.S.A- 11203.

Idiopathic inflammatory myopathy (IIM) is a rare autoimmune myopathy that includes polymyositis, dermatomyositis, inclusion body myositis and autoimmune necrotizing myositis. Cardiac involvement was considered a rare occurrence in IIM however, recent reports suggests that cardiac involvement is a common feature and portends poor prognosis as it is usually encountered in advanced disease. IIM leads to myocarditis with subsequent development of myocardial fibrosis, cardiac conduction system disease and cardiomyopathy resulting in both systolic and diastolic heart failure. Read More

Clin Rheumatol 2018 Sep 14. Epub 2018 Sep 14.

Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Kanagawa, 216-8511, Japan.

To evaluate the efficacy and tolerability of mycophenolate mofetil (MMF) with or without calcineurin inhibitors (CNIs) in patients with inflammatory myopathy taking prednisolone, but refractory to conventional immunosuppressive therapy. The records of patients with inflammatory myopathy who had previously failed treatment with at least one immunosuppressant were retrospectively evaluated. We selected patients treated with MMF and divided them into two groups depending on whether or not there was concomitant use of CNIs. Read More

Am Fam Physician 2018 Aug;98(3):164-170

Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Patients with a suspected connective tissue disorder should undergo serologic testing to confirm the diagnosis and, in some cases, to monitor disease activity and predict flares. Patients with suspected systemic lupus erythematosus should be tested for antinuclear antibodies. However, antinuclear antibodies are not specific and may be present in many other connective tissue disorders and nonrheumatologic diseases. Read More

JAMA Neurol 2018 Dec;75(12):1528-1537

Centre de Recherche en Myologie, Unité Mixte de Recherche Scientifique 974, Université Pierre et Marie Curie, Institut National de la Santé et de la Recherche Médicale, Paris, France.

Importance: Idiopathic inflammatory myopathies are heterogeneous in their pathophysiologic features and prognosis. The emergence of myositis-specific autoantibodies suggests that subgroups of patients exist.

Objective: To develop a new classification scheme for idiopathic inflammatory myopathies based on phenotypic, biological, and immunologic criteria. Read More

J Rheumatol 2018 Dec 1;45(12):1689-1695. Epub 2018 Sep 1.

From the Division of Rheumatology, Allergy, and Immunology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Objective: Systemic autoimmune rheumatic diseases (SARD) are associated with an increased risk of premature cardiovascular disease (CVD) and all-cause mortality. We examined the potential survival benefit of statin use among patients with SARD in a general population setting.

Methods: We conducted an incident user cohort study using a UK general population database. Read More

Clin Radiol 2018 Dec 30;73(12):1057.e13-1057.e18. Epub 2018 Aug 30.

Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China. Electronic address:

Aim: To explore the T2-mapping signal characteristics of the thigh muscles in patients with dermatomyositis/polymyositis (DM/PM) and to investigate the correlation between thigh muscle T2 values, clinical parameters, and serum creatinine kinase (CK).

Materials And Methods: Forty-two patients with DM/PM proven by diagnostic criteria were enrolled in the study along with 13 healthy control subjects. Both T2-mapping and conventional magnetic resonance imaging (MRI) images were obtained in the thigh musculature of all subjects. Read More

Fukushima J Med Sci 2018 ;64(2):89-94

Department of Rheumatology, Fukushima Medical University School of Medicine.

Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) resulting in high mortality. Here we report a 51-year-old Japanese woman with anti-MDA5 antibody-positive hypomyopathic dermatomyositis (DM) who developed RP-ILD. She developed respiratory failure and pneumomediastinum, however her RP-ILD responded favorably to the combined immunosuppressive treatments consisting of steroids, intravenous cyclophosphamide and tacrolimus. Read More

Arthritis Res Ther 2018 Aug 29;20(1):188. Epub 2018 Aug 29.

Division of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.

Background: Polymyositis (PM) and dermatomyositis (DM) are two distinct subgroups of idiopathic inflammatory myopathies, a chronic inflammatory disorder clinically characterized by muscle weakness and inflammatory cell infiltrates in muscle tissue. In PM, a major component of inflammatory cell infiltrates is CD8+ T cells, whereas in DM, CD4+ T cells, plasmacytoid dendritic cells, and B cells predominate. In this study, with the aim to differentiate involvement of CD4+ and CD8+ T-cell subpopulations in myositis subgroups, we investigated transcriptomic profiles of T cells from peripheral blood of patients with myositis. Read More

Am J Case Rep 2018 Aug 28;19:1025-1029. Epub 2018 Aug 28.

Department of Pulmonary and Critical Care Medicine, China Medical University Hospital, Taichung, Taiwan.

BACKGROUND Aortoesophageal fistula is a rare etiology of serious gastrointestinal bleeding. Most aortoesophageal fistulas resulted from thoracic aortic aneurysms, foreign bodies, or esophageal malignancy. To our knowledge, spontaneous aortoesophageal fistula due to dermatomyositis and high dose steroid therapy has not been reported. Read More

Respir Investig 2018 Nov 24;56(6):464-472. Epub 2018 Aug 24.

Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, Asahi-machi 67, Kurume, Fukuoka 830-0011, Japan. Electronic address:

Background: Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab) is associated with fatal rapidly progressive interstitial lung disease (RP-ILD) in patients with dermatomyositis (DM). We attempted to clarify whether anti-MDA5-Ab is associated with long-term outcomes in patients with DM-ILD.

Methods: Thirty-six patients with DM-ILD were retrospectively analyzed for their serum anti-MDA5-Ab by using an enzyme-linked immunosorbent assay. Read More

Int J Rheum Dis 2018 Aug;21(8):1627-1633

Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.

Aim: This study aimed to follow up patients with polymyositis (PM) and/or dermatomyositis (DM) to determine survival rate, pattern of disease, response to treatment, malignancy incidence and poor prognostic factors (PPFs).

Method: A total of 76 patients with PM (n = 47) and/or DM (n = 29) based on Bohan and Peter diagnostic criteria referred to the Imam-Reza Hospital were followed up from 2004 to 2016. The follow-up period was considered from diagnosis to patient's death or last visit. Read More

Int J Rheum Dis 2018 Aug;21(8):1619-1626

Department of Rheumatology, Beijing Key Lab for Immune-Mediated Inflammatory Diseases, China-Japan Friendship Hospital, Beijing, China.

Objectives: Previous association studies have identified genetic variants in the human leukocyte antigen (HLA) complex as substantial risk factors for idiopathic inflammatory myopathies (IIMs). However, a great number of genes are located in the HLA region, and thus fine mapping is quite necessary.

Methods: Targeted capture sequencing were performed on the whole HLA region in 42 IIM patients and 24 healthy controls. Read More

Clin Rheumatol 2018 Aug 25. Epub 2018 Aug 25.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

The aim of our study was to investigate the association between single nucleotide polymorphisms (SNPs) in the BANK1 gene and polymyositis/dermatomyositis (PM/DM) in a Chinese Han population. In total, 363 PM patients, 654 DM patients, and 1280 healthy controls were recruited and genotyped using the Sequenom MassArray system. A significant allele association was observed in rs3733197 among the PM/DM patients (OR 0. Read More

Clin Rheumatol 2018 Aug 25. Epub 2018 Aug 25.

Institute for Systemic Autoimmune and Neurological Diseases, Athens, Greece.

Myositis-specific (MSAs) or-associated autoantibodies (MAAs) have been linked to particular clinical phenotypes of idiopathic inflammatory myopathies (IIM) and appear to aid diagnosis. The objective of this study was to analyze the prevalence of MSAs and MAAs and their possible clinical associations in Greek IIM patients. This study comprised 95 IIM patients classified based on the 2017 EULAR/ACR classification criteria. Read More

Medicine (Baltimore) 2018 Aug;97(34):e11775

Department of Rheumatism and Immunology, Affiliated Hospital of Qinghai University, Xining.

This study aims to explore the roles of cysteine-rich protein 61 (Cyr61/CCN1), connective tissue growth factor (CTGF/CCN2) and vascular endothelial growth factor (VEGF) in the vascular process of polymyositis (PM)/dermatomyositis (DM).Real-time quantitative polymerase chain reaction was used to determine the mRNA expression of Cyr61, CTGF, and VEGF in muscle tissues of initially treated PM/DM patients and controls. Enzyme-linked immunosorbent assay (ELISA) was used to determine the serum levels of Cyr61, CTGF, and VEGF of initially treated PM/DM patients before and after treatment. Read More

Zhonghua Jie He He Hu Xi Za Zhi 2018 Aug;41(8):616-621

Department of Respiratory Medicine, Drum Tower, Clinical Medical College of Nanjing Medical University, Nanjing 210008, China.

To investigate the profile and clinical significance of myositis-specific antibody spectrum (MSAs) in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Sera from 74 patients with PM/DM-ILD, 29 patients with SLE and 32 healthy controls were collected and Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs . The clinical data of all patients were collected from medical records. Read More

Rev Assoc Med Bras (1992) 2018 Apr;64(4):311-314

Ambulatory of neuromuscular diseases.

As the celiac disease (CD), the non-celiac gluten sensitivity (NCGS) has also been associated with several autoimmune manifestations. It is rarely associated with myasthenia gravis (MG). This paper shall introduce the case of a young female patient, initially presenting a peripheral neuropathy framework. Read More

Lancet Neurol 2018 Sep;17(9):816-828

Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA; Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Inflammatory myopathies, collectively known as myositis, are heterogeneous disorders characterised by muscle inflammation, and frequently accompanied by extramuscular manifestations that affect the skin, lung, and joints. Patients with inflammatory myopathies were previously classified as having dermatomyositis if characteristic rashes accompanied the muscle involvement, and as having polymyositis if no rashes were present. Five main types of inflammatory myopathies are now widely recognised: dermatomyositis, immune-mediated necrotising myopathy, sporadic inclusion-body myositis, overlap myositis (including antisynthetase syndrome), and polymyositis. Read More

Br J Dermatol 2018 Aug 18. Epub 2018 Aug 18.

Department of Dermatology, Faculty of Medicine, University of Tsukuba, Tsukuba, Japan.

Background: Myositis-specific autoantibodies (MSAs) are associated with unique clinical subsets in polymyositis/dermatomyositis (PM/DM). Autoantibodies against transcriptional intermediary factor (TIF)-1γ and TIF-1α are known to be MSAs. Previously, we reported that TIF-1β is also targeted in patients with DM with or without concomitant anti-TIF-1α/γ antibodies. Read More

RMD Open 2018 25;4(2):e000661. Epub 2018 Jul 25.

Department of Rheumatology, University Hospitals Leuven, Leuven, Belgium.

Objective: As rheumatoid factor (RF) and anticitrullinated protein antibodies (ACPAs) are not routinely tested in idiopathic inflammatory myositis (IIM), little is known about their prevalence and clinical implications in this patient group. In antisynthetase syndrome (ASS), presence of ACPA is reportedly associated with more severe and erosive arthritis. We aim to retrospectively determine the prevalence of RF and ACPA in a cross-sectional cohort of 121 patients diagnosed with IIM and to assess clinical associations. Read More

Medicine (Baltimore) 2018 Aug;97(33):e11586

Department of Rheumatology, Aerospace Center Hospital, Beijing, PR China.

Rationale: Dermatomyositis is an idiopathic inflammatory myopathy with specific cutaneous manifestations, which is closely associated with malignancy. However, the exact mechanism remains elusive. Even less is known about dermatomyositis with hepatocellular carcinoma (HCC). Read More

Rheumatology (Oxford) 2018 Dec;57(12):2149-2157

Department of Medicine, Unit of Rheumatology, Karolinska Institutet, Solna, Sweden.

Objectives: To investigate the expression of IL-18 in symptomatic and asymptomatic muscle tissues of patients with PM and DM and the effects of conventional immunosuppressive treatment on such expression.

Methods: Two cohorts of patients were included in this study. The first cohort consisted of 10 new-onset myositis patients. Read More

Prog Lipid Res 2018 10 9;72:1-17. Epub 2018 Aug 9.

Orthopaedic Research & Biotechnology, The Children's Hospital at Westmead, Westmead, NSW, Australia.; Discipline of Paediatrics & Child Heath, Faculty of Medicine, University of Sydney, Camperdown, NSW, Australia. Electronic address:

Lipid storage myopathies (LSMs) are a heterogeneous group of genetic disorders that present with abnormal lipid storage in multiple body organs, typically muscle. Patients can clinically present with cardiomyopathy, skeletal muscle weakness, myalgia, and extreme fatigue. An early diagnosis is crucial, as some LSMs can be managed by simple nutraceutical supplementation. Read More

AJR Am J Roentgenol 2018 Oct 7;211(4):861-866. Epub 2018 Aug 7.

1 Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Dongcheng District, Beijing, China.

Objective: Diffusion tensor imaging (DTI) has long been proposed as a potential tool for quantitatively evaluating muscle lesions of dermatomyositis (DM) and polymyositis (PM), but adapting DTI to thigh muscles requires minimizing acquisition time. We investigated the clinical feasibility of using SMS-EPI-DTI (simultaneous multislice [SMS] accelerated echo planar imaging [EPI] DTI) to image and analyze the anisotropic diffusion characteristics of thigh muscles in patients with DM or PM.

Subjects And Methods: Both thighs of 20 patients (seven patients with DM and 13 patients with PM) and 10 healthy control subjects were prospectively scanned using SMSEPI-DTI. Read More

Pediatr Radiol 2018 08 4;48(9):1348-1363. Epub 2018 Aug 4.

Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON,, M5G 1X8, Canada.

Whole-body MRI is increasingly utilized for assessing oncologic and non-oncologic diseases in infants, children and adolescents. Focusing on the non-oncologic indications, this review covers technical elements required to perform whole-body MRI, the advantages and limitations of the technique, and protocol modifications tailored to specific indications. Rheumatologic diseases account for the majority of non-oncologic whole-body MRI performed in pediatric patients at the author's institution. Read More