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Rheumatol Int 2019 Feb 21. Epub 2019 Feb 21.

Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Tochigi, Japan.

The objective was to investigate the clinical and histological features of liver dysfunction in patients with polymyositis (PM) or dermatomyositis (DM).A total of 115 patients (38 with PM and 77 with DM), who were admitted to our hospital between 2001 and 2012, were retrospectively reviewed. Liver dysfunction was defined as an alanine transaminase (ALT) level ≥ 60 U/l and a disproportionate ALT elevation relative to the creatine kinase level. Read More

Clin Ter 2019 Jan-Feb;170(1):e55-e60

Department of Clinical and Experimental Medicine, University of Pisa, Italy.

The chemokine monokine induced by interferon (IFN)-γ (MIG) is expressed in idiopathic inflammatory myopathies muscle. Abundant expression of MIG was observed on macrophages and T cells surrounding and invading non-necrotic muscle fibers in polymyositis and in inclusion-body myositis and in T cells in perimysial infiltrates of dermatomyositis. MIG is also localized to blood vessel endothelial cells in all inflammatory and normal muscle tissues and it exerts its biological effects mainly via binding to the chemokine (C-X-C motif) receptor (CXCR)3. Read More

Eur J Med Res 2019 Feb 14;24(1):12. Epub 2019 Feb 14.

Department of Immunology, No. 983 of the Chinese People's Liberation Army Joint Support Force, No. 60 of Huangwei Street, Hebei District, Tianjin, 300142, China.

Objective: This study aims to detect serum levels of monocyte chemoattractant protein-1 (MPC-1) and transforming growth factor-β1 (TGF-β1) in polymyositis/dermatomyositis (PM/DM) patients complicated with interstitial lung disease (ILD), to reveal the significance of the changes in these levels in the pathogenesis of PM/DM complicated with ILD.

Methods: Serum MCP-1 and TGF-β1 levels in PM/DM patients complicated with ILD, patients with pulmonary infections and normal controls (n = 30, each) were detected using enzyme-linked immunosorbent assay (ELISA), and the correlation between PM/DM complicated with ILD and serum MCP-1 and TGF-β1 levels was analyzed.

Results: Serum MCP-1 and TGF-β1 levels were both higher in PM/DM patients complicated with ILD compared with patients with pulmonary infections and normal controls. Read More

Ann Rheum Dis 2019 Feb 13. Epub 2019 Feb 13.

Department Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden.

J Clin Rheumatol 2019 Feb 1. Epub 2019 Feb 1.

From the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Objectives: Idiopathic inflammatory myositis (IIM) commonly affects women in the childbearing age group. Both disease activity and immunosuppressants used may have adverse effects on fertility and outcomes of pregnancy. We explored these outcomes in a retrospective cohort of women with IIM. Read More

Joint Bone Spine 2019 Jan 31. Epub 2019 Jan 31.

Service de rhumatologie, Hôpitaux Universitaires de Strasbourg, 67098 Strasbourg, France; Centre de Référence des Maladies Autoimmunes Rares de l'Est & Sud Ouest, 33000 Bordeaux, France; Fédération de Médecine Translationnelle de Strasbourg, Université de Strasbourg, 67081 Strasbourg, France.

Skeletal muscle inflammation is the feature shared by all forms of myositis. However, the muscle damage ranges in severity from asymptomatic to responsible for severe weakness. In addition, myositis usually occurs as a systemic disease that affects multiple organs. Read More

Prostate Cancer Prostatic Dis 2019 Jan 28. Epub 2019 Jan 28.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.

Background: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases.

Methods: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases. Read More

Cytokine 2019 Apr 23;116:13-20. Epub 2019 Jan 23.

Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic. Electronic address:

Background: S100A11 (calgizzarin), a member of the S100 family, is associated with oncogenesis, inflammation and myocardial damage. Our aim was to analyse S100A11 in idiopathic inflammatory myopathies (IIMs) and its association with disease activity features and cancer development.

Methods: S100A11 in muscle was determined by immunohistochemistry in polymyositis (PM), dermatomyositis (DM), myasthenia gravis (MG) and in subjects without autoimmune inflammatory disease (HC). Read More

Adv Rheumatol 2019 Jan 22;59(1). Epub 2019 Jan 22.

Disciplina de Reumatologia, Faculdade de Medicina, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3° andar, sala 3150 - Cerqueira César, Sao Paulo, CEP: 01246-903, Brazil.

Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM).

Main Body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Read More

Nucl Med Commun 2019 Jan 18. Epub 2019 Jan 18.

Nuclear Medicine Department, Lady Davis Institute, Jewish General Hospital.

Background: To compare the performance of fluorine-18-fluorodeoxyglucose (F-FDG) PET/computed tomography (CT) and conventional tests for cancer screening in autoimmune inflammatory myopathy (AIM) patients.

Patients And Methods: We carried out a retrospective cohort study of AIM patients from one academic center in Montreal, Canada, classified using myositis-specific antibodies, who underwent F-FDG PET/CT between April 2005 and February 2018 and were followed up on average 3.5±2. Read More

Adv Rheumatol 2018 Oct 22;58(1):34. Epub 2018 Oct 22.

Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3 andar, sala 3150 - Cerqueira César, CEP 01246-903, Sao Paulo, Brazil.

Background: Currently, there are only few studies (mostly case reports or case series) on mycophenolate mofetil (MMF) in patients with systemic autoimmune myopathies (SAM). Therefore, the goal of the present study was to evaluate the safety and efficacy of MMF (monotherapy or coadjuvant drug) in a specific sample of patients with refractory SAM: dermatomyositis, polymyositis, anti-synthetase syndrome or clinically amyopathic dermatomyositis.

Methods: A case series including 20 consecutive adult patients with refractory SAM from 2010 to 2016 was conducted. Read More

Adv Rheumatol 2018 Sep 18;58(1):31. Epub 2018 Sep 18.

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

Background: Interpretation of rituximab efficacy for refractory idiopathic inflammatory myopathies (IIM) is hampered by the absence of a uniform definition of refractory myositis and clinical response. Therefore, rigorous criteria of refractoriness, together with a homogenous definition of clinical improvement, were used to evaluate rituximab one-year response.

Methods: A retrospective cohort study including 43 IIM (15 antisynthetase syndrome, 16 dermatomyositis, 12 polymyositis) was conducted. Read More

Adv Rheumatol 2018 May 24;58(1). Epub 2018 May 24.

Division of Rheumatology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, Av. Dr. Arnaldo, 455, 3° andar, sala 3150 - Cerqueira César, Sao Paulo, 01246-903, Brazil.

Systemic autoimmune myopathies (SAMs) are a heterogeneous group of rare systemic autoimmune diseases that primarily affect skeletal muscles. Patients with SAMs show progressive skeletal muscle weakness and consequent functional disabilities, low health quality, and sedentary lifestyles. In this context, exercise training emerges as a non-pharmacological therapy to improve muscle strength and function as well as the clinical aspects of these diseases. Read More

Curr Health Sci J 2018 Jul-Sep;44(3):316-321. Epub 2018 Jul 15.

Department of Pharmacology, University of Medicine and Pharmacy of Craiova, Romania.

Glucosteroids (GS) are widely used drugs for various inflammatory pathologies (Nephrotic syndrome, Proliferative glomerulonephritis, Extramembrane glomerulonephritis, Nephropathy of the Nodous Poliarterita (PAN), Nephropathy from purple Henoch-Schonlein, lupus nephropathy (LN), Acute adrenal insufficiency Waterhouse-Friederichsen, Chronic adrenal insufficiency Addison, Systemic Lupus Erythematosus (SLE), Polymyositis and dermatomyositis, Chronic granulomatosis, Crohn's disease, Hemorrhagic rectocolitis, Hemolytic anemias, Acute leukemias and chronic lymphocytic leukemia, Hodgkin's lymphoma). Although they are prescribed for their anti-inflammatory and immunosuppressive properties, they also have many side effects, hyperglycemia being one of the most common and representative, which is why these drugs need careful monitoring when administered over the long term. This paper presents the case of a 39 year old patient diagnosed with systemic lupus erythematosus (SLE) with class IV lupus nephropathy (LN) who developed numerous complications due to the pathogenic side effects: diabetes, amenorrhea, recurrent infections, and depression. Read More

J Rheumatol 2019 Jan 15. Epub 2019 Jan 15.

From the Second Division, Department of Internal Medicine, and the Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu; Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan. H. Hozumi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Fujisawa, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; R. Nakashima, Research Associate, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; H. Yasui, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; Y. Suzuki, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Kono, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; M. Karayama, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; K. Furuhashi, Research Associate, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Enomoto, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; N. Inui, Assistant Professor, MD, PhD, Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine; Y. Nakamura, Lecturer, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine; T. Mimori, Professor, MD, PhD, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University; T. Suda, Professor, MD, PhD, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine. Address correspondence to Dr. H. Hozumi, Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashiku, Hamamatsu 431-3192, Japan. E-mail: or Accepted for publication October 10, 2018.

Objective: The optimal treatment strategy for anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis-associated interstitial lung disease (anti-ARS-PM/DM-ILD) is yet to be established. We aimed to evaluate the efficacy of glucocorticoids and calcineurin inhibitors (CNI) in patients with ARS-PM/DM-ILD.

Methods: Progression-free survival (PFS) and overall survival rates were retrospectively evaluated in 32 consecutive patients with ARS-PM/DM-ILD. Read More

Clin Rheumatol 2019 Jan 15. Epub 2019 Jan 15.

Department of Rheumatology, Beijing Friendship Hospital, Capital Medical University, No. 95 Yongan Road, Xicheng District, Beijing, China.

Objectives: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of the TNF super-family, which is involved in the regulation of immune response and pathogenesis of autoimmune diseases, including polymyositis (PM) and dermatomyositis (DM). In this study, we examined the level and origin of serum-soluble TRAIL (sTRAIL) in patients with PM and DM and analyzed its association with disease activity and clinical features.

Method: 11 PM patients, 33 DM patients, and 20 healthy controls were enrolled in this study. Read More

Arch Rheumatol 2018 Sep 15;33(3):376-380. Epub 2018 Jan 15.

Department of General Internal Medicine, Rakuwakai Otowa Hospital, Kyoto, Japan.

Interstitial lung disease in polymyositis and dermatomyositis is a serious complication, associated with poor prognosis. In this article, we describe two cases with histological findings of organizing pneumonia, based on transbronchial lung biopsy. One is a 66-year-old female patient with clinically amyopathic dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody, and another is a 61-year-old female patient with polymyositis with anti-Jo-1 antibody. Read More

Clin Exp Rheumatol 2018 Dec 20. Epub 2018 Dec 20.

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

Objectives: To assess serum interleukin (IL)-17A levels in patients with dermatomyositis (DM) and polymyositis (PM) and correlate them with the demographic, clinical, laboratory and therapeutic data of these diseases.

Methods: This was a cross-sectional, single-centre study that included defined DM and PM patients who were age-, gender- and ethnicity-matched to healthy individuals. Serum IL-17A analysis, as well as analysis for other cytokines (IL-6, TNFα and IFNγ), was performed by multiplex immunoassay. Read More

J Neurol Sci 2019 Feb 31;397:123-128. Epub 2018 Dec 31.

Department of Clinical Laboratory, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, PR China. Electronic address:

Aim: The prevalence and diagnostic values of myositis-specific autoantibodies (MSAs) and myositis-associated autoantibodies (MAAs) in dermatomyositis/polymyositis (DM/PM) were studied.

Method: A commercial immunoblot assay with 16 autoantigens was used to detect MSAs and MAAs in serum samples from 130 DM/PM patients, 100 disease controls, and 50 healthy subjects.

Results: The prevalence of anti-Jo-1, anti-MDA5, anti-TIF1γ, anti-Mi-2α, and anti-Mi-2β was significantly higher in DM/PM than in other connective-tissue diseases (CTDs). Read More

Curr Treatm Opt Rheumatol 2018 23;4(4):289-298. Epub 2018 Nov 23.

Department of NVS, Huddinge, Karolinska Institutet and Department of Medicine, Karolinska Institutet, Solna and Function Area Occupational Therapy and Physical Therapy, Karolinska University Hospital, SE-171 76 Stockholm, Sweden.

Purpose Of The Review: A growing body of evidence supports exercise as a very important part of the treatment for adult patients with idiopathic inflammatory myopathies (IIM). This review mainly focuses on exercise studies published during the last 2 years in adult myositis.

Recent Findings: During the last couple of years, new publications present further evidence for intensive endurance exercise as an anti-inflammatory treatment inducing muscle growth and improving mitochondrial function compared a non-exercising control group. Read More

Pediatr Rheumatol Online J 2018 Dec 29;16(1):84. Epub 2018 Dec 29.

Division of Pediatric Rheumatology, Department of Pediatrics, St. Louis, MO, USA.

Background: Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis.

Method: The CARRA legacy registry recruited pediatric rheumatology patients from 55 centers across North America from 2010 through 2014, including over 650 subjects with Juvenile Dermatomyositis. Read More

Cardiol Rev 2018 Dec 21. Epub 2018 Dec 21.

Idiopathic inflammatory myopathies are a group of autoimmune diseases that are characterized by muscle inflammation resulting in elevated muscle enzyme release and distinctive biopsy findings. This group of conditions includes polymyositis, dermatomyositis, inclusion body myositis and necrotizing autoimmune myopathy. Although they have many similarities, the inflammatory myopathies differ in their clinical, pathological and treatment realms. Read More

Neuromuscul Disord 2019 Jan 22;29(1):5-13. Epub 2018 Nov 22.

Research Institute of Neuromuscular and Neurodegenerative Diseases and Department of Neurology, Qilu Hospital, Shandong University, Jinan, China; Mitochondrial Medicine Laboratory, Qilu Hospital (Qingdao), Shandong University, Qingdao, China; Brain Science Research Institute, Shandong University, Jinan, China. Electronic address:

Anti-mitochondrial antibodies, the hallmark of primary biliary cirrhosis, have been detected in many patients with idiopathic inflammatory myopathies and these anti-mitochondrial-antibody-associated idiopathic inflammatory myopathies frequently show unique characteristics. We detected anti-mitochondrial antibodies in Chinese idiopathic inflammatory myopathy and summarized the clinical findings of these anti-mitochondrial-antibody-positive patients. Of 136 patients, seven (5. Read More

Reumatismo 2018 12 20;70(4):257-258. Epub 2018 Dec 20.

Rheumatology Unit, University of Modena and Reggio Emilia, Modena.

A 57-year-old woman with a diagnosis of antisynthetase syndrome (ASSD) underwent a nailfold videocapillaroscopy (NVC) showing a scleroderma pattern. Alterations in capillary morphology have been reported in adults with inflammatory myositis (IM) but only recently have the differences in NVC findings between these two diseases been established. ASSD is currently classified as a subset of IM, for which reason only a few studies in literature evaluate its specific hallmarks, showing nonspecific features of NVC in patients with polymyositis and dermatomyositis (DM) and antisynthetase antibodies. Read More

Mod Rheumatol 2019 Jan 19;29(1):1-19. Epub 2018 Dec 19.

l 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

J Biophotonics 2018 Dec 18:e201800354. Epub 2018 Dec 18.

Department of Dermatology and Allergology, Juntendo University Graduate School of Medicine, Tokyo, Japan.

In finger vein authentication technology, near-infrared rays penetrate the finger and are absorbed by the hemoglobin in blood. The veins appear as dark areas. The finger vein pattern images of patients with various diseases were acquired; a new evaluation method applying image processing technique ("E value") was developed, and it was examined whether the patterns have any characteristics differentiating them from those of healthy volunteers. Read More

J Dermatol 2019 Jan 18;46(1):e1-e18. 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 2019 Mar 14;39(3):577-581. 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

Medicine (Baltimore) 2018 Dec;97(49):e13563

Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.

Rationale: An association between inflammatory myopathy and malignancy has been recognized particularly in patients positive for anti-transcription intermediary factor 1γ (TIF1γ) antibody. We report a case of anti-TIF1γ antibody positive dermatomyositis (DM) associated with thymic carcinoma which radiographically mimicked benign tumor.

Patient Concerns: A 72-year-old man presented typical characteristic cutaneous manifestations and proximal muscle weakness with elevated levels of myogenic enzymes. 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 and Centre de Recherche des Cordeliers, Equipe-Immunopathologie et Immunointervention Thérapeutique, Sorbonne Université, Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address:

Background: Therapeutic normal IgG or intravenous immunoglobulin (IVIG) exerts anti-inflammatory effects through several mutually nonexclusive mechanisms. Recent data in mouse models of autoimmune disease suggest that IVIG induces IL-4 in basophils by enhancing IL-33 in SIGN-related 1-positive innate cells. However, translational insight on these data is lacking. Read More

Rheumatol Int 2019 Feb 5;39(2):311-316. 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 2019 Jan 9;89(1):e12732. Epub 2018 Dec 9.

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 2019 Feb 5;22(2):314-320. 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

Br J Radiol 2019 Feb 14;92(1094):20180715. Epub 2018 Nov 14.

1 Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology , Wuhan , China.

Objective:: To explore and confirm feasibility the T maps and readout-segmented echoplanar imaging (rs-EPI) diffusion-weighted imaging (DWI) characteristics of the thigh muscles for dermatomyositis (DM) patients.

Methods:: This study was approved by the University Institutional Review and written informed consent was obtained from all subjects before enrollment (trial registration number: TJ-C20121221). 28 patients with DM proven by diagnostic criteria were enrolled in the study along with 9 healthy control subjects. 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 10;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

BMJ Case Rep 2018 Oct 7;2018. Epub 2018 Oct 7.

Dermatology, Cliniques universitaires Saint-Luc, Bruxelles, Belgium.

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