11,936 results match your criteria Dermatomyositis

Gene Expression Profiles of Treatment Response and Non-Response in Children With Juvenile Dermatomyositis.

ACR Open Rheumatol 2022 May 26. Epub 2022 May 26.

Duke University, Durham, North Carolina.

Objective: The study objective was to identify differences in gene expression between treatment responders (TRs) and treatment non-responders (TNRs) diagnosed with juvenile dermatomyositis (JDM).

Methods: Gene expression analyses were performed using whole blood messenger RNA sequencing in patients with JDM (n = 17) and healthy controls (HCs; n = 10). Four analyses were performed (A1-4) comparing differential gene expression and pathways analysis exploiting the timing of sample acquisition and the treatments received to perform these comparative analyses. Read More

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Altered Gut Microbiome in Patients With Dermatomyositis.

ACR Open Rheumatol 2022 May 26. Epub 2022 May 26.

University of California, Los Angeles School of Medicine, Los Angeles.

Objective: The study objective was to compare the microbial composition of patients with dermatomyositis (DM) and healthy controls (HCs) and determine whether microbial alterations are associated with clinical manifestations of DM.

Methods: The 16S ribosomal RNA gene sequencing was performed on fecal samples from patients with DM and HCs. Microbial composition and diversity were compared between subjects with DM and HCs and in association with several DM-specific clinical variables, including myositis-specific autoantibodies (MSAs). Read More

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Mortality Risk Stratification Using Cluster Analysis in Patients With Myositis-Associated Interstitial Lung Disease Receiving Initial Triple-Combination Therapy.

Front Med (Lausanne) 2022 9;9:883699. Epub 2022 May 9.

Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan.

Objective: To stratify patients with polymyositis/dermatomyositis-associated interstitial lung disease (ILD) who were initially treated with an intensive regimen consisting of high-dose corticosteroids, a calcineurin inhibitor, and intravenous cyclophosphamide (triple-combo therapy) into subgroups based on mortality outcomes by a cluster analysis using a large-scale multicenter retrospective cohort of Japanese patients with myositis-associated ILD (JAMI).

Methods: Two-step cluster analysis of preclustering and subsequent hierarchical clustering was conducted in 185 patients who received triple-combo therapy in an unbiased manner. Initial predictors for mortality previously reported in patients with myositis-associated ILD were used as variables and included age, sex, disease duration, classification of myositis, requirement of supplemental oxygen, anti-aminoacyl tRNA synthetase (ARS) antibody, anti-melanoma differentiation-associated gene 5 (MDA5) antibody, and serum levels of C-reactive protein (CRP) and Krebs von den Lungen-6 (KL-6). Read More

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Successfully treatment with Dupilumab for systemic contact dermatitis following hair dye in a patient with dermatomyositis.

J Cosmet Dermatol 2022 May 25. Epub 2022 May 25.

Department of Allergy, Shandong Provincial Qianfoshan Hospital, The First Affiliated Hospital of Shandong First Medical University, Shandong Institute of Respiratory Diseases, Jinan, China, 250013.

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Anti-nuclear matrix protein 2+ juvenile dermatomyositis with severe skin ulcer and infection: A case report and literature review.

World J Clin Cases 2022 Apr;10(11):3579-3586

Department of Rheumatology and Immunology; Ministry of Education Key Laboratory of Child Development and Disorders; National Clinical Research Center for Child Health and Disorders (Chongqing); China International Science and Technology Cooperation base of Child Development and Critical Disorders; Children's Hospital of Chongqing Medical University, Chongqing 400014, China.

Background: Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy that occurs in childhood. It is characterized by muscle weakness and a characteristic rash. Previous literature reports have rarely described JDM with severe skin ulcers and infections. Read More

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Genetics and Autoimmunity: Two Sides of the Same Coin or an Epiphenomenon?

Mediterr J Rheumatol 2022 Mar 31;33(1):63-67. Epub 2022 Mar 31.

Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.

Distinguishing hereditary forms of myopathy from certain forms of inflammatory myopathy can be challenging. We present 3 cases where a certain degree of overlap was observed between genetics and autoimmunity. A child with juvenile dermatomyositis where heterozygosity for a pathogenic mutation implicated in LGMD1C resulted in a delayed diagnosis. Read More

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Identification of hub biomarkers and immune cell infiltration in polymyositis and dermatomyositis.

Aging (Albany NY) 2022 May 24;14(undefined). Epub 2022 May 24.

Department of Clinical Laboratory, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.

Objective: Polymyositis (PM) and dermatomyositis (DM) are heterogeneous disorders. However, the etiology of PM/DM development has not been thoroughly clarified.

Methods: Gene expression data of PM/DM were obtained from Gene Expression Omnibus. Read More

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Preclinical ocular microvascular changes in juvenile dermatomyositis: A pilot optical coherence tomography angiography study.

Microvasc Res 2022 May 20:104382. Epub 2022 May 20.

Kocaeli University, Department of Ophthalmology, Kocaeli, Turkey.

Objectives: To evaluate the macular and optic nerve head (ONH) vascular density, foveal avascular zone area, and outer retina and choriocapillaris flow in juvenile dermatomyositis (JDM) using optical coherence tomography angiography (OCTA).

Methods: Ten eyes of 10 patients with JDM and 15 age and sex-matched healthy controls were investigated in this prospective, cross-sectional study. The superficial capillary plexus (SCP) and deep capillary plexus (DCP), ONH, foveal avascular zone (FAZ) parameters, the flow area of the outer retina, and choriocapillaris were evaluated using OCTA. Read More

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Prevalence and characteristics of inflammatory rheumatic diseases in patients with thalassemia.

Ann Hematol 2022 May 23. Epub 2022 May 23.

Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, 50200, Chiang Mai, Thailand.

Reports of inflammatory rheumatic diseases (IRD) in thalassemia are limited. This study aimed to determine the prevalence and clinical characteristics of IRD in patients with thalassemia disease. Consecutive adult patients with thalassemia disease, confirmed by hemoglobin typing, attending the Hematology Clinic between June 2019 and May 2021 were invited to join this study. Read More

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pneumonia after total hip arthroplasty in a dermatomyositis patient: A case report.

World J Clin Cases 2022 Apr;10(10):3313-3320

Department of Orthopaedic Surgery, Beijing Key Lab Immune-Mediated Inflammatory Diseases, China-Japan Friendship Hospital, Beijing 100029, China.

Background: pneumonia (PJP) is a serious opportunistic infection that occurs mostly in patients with immunodeficiency and long-term immunosuppressive therapy. In non-human immunodeficiency virus-infected patients, the most important risk factor for PJP is the use of glucocorticoids in combination with other immunosuppressive treatments. The management of glucocorticoids during the perioperative period in patients with dermatomyositis requires special care. Read More

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Screening for Anti-HMGCR Antibodies in a Large Single Myositis Center Reveals Infrequent Exposure to Statins and Diversiform Presentation of the Disease.

Front Immunol 2022 4;13:866701. Epub 2022 May 4.

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

Background: The objective of this study is to assess the frequency of autoantibodies against 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) in a single center myositis cohort and to analyze associations with statin exposure, clinical features, and outcome of disease course.

Methods: A total of 312 patients with idiopathic inflammatory myopathies (IIMs) followed at the rheumatology clinic, Karolinska University Hospital, were identified in the Euromyositis registry between 1988 and 2014 and were classified according to the 2017 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) criteria. Available serum samples were analyzed for anti-HMGCR autoantibodies by ELISA. Read More

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Interstitial Lung Disease in Dermatomyositis Without Myositis-Specific and Myositis-Associated Autoantibodies: Study of a Series of 72 Patients From a Single Cohort.

Front Immunol 2022 6;13:879266. Epub 2022 May 6.

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

Objective: The clinical features of interstitial lung disease (ILD) in patients with dermatomyositis (DM) and negative myositis autoantibodies had not been exactly demonstrated previously. This study aimed to describe and expand the phenotype of interstitial lung disease (ILD) in this cohort of patients.

Methods: A total of 1125 consecutive Chinese patients with idiopathic inflammatory myopathies (IIM) between 2006 and 2020 were screened retrospectively. Read More

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Cardiovascular Risk in Myositis Patients Compared to the General Population: Preliminary Data From a Single-Center Cross-Sectional Study.

Front Med (Lausanne) 2022 3;9:861419. Epub 2022 May 3.

Institute of Rheumatology, Prague, Czechia.

Background: Idiopathic inflammatory myopathies (IIM) are associated with systemic inflammation, limited mobility, and glucocorticoid therapy, all of which can lead to metabolism disturbances, atherogenesis, and increased cardiovascular (CV) risk. The aim of this study was to assess the CV risk in IIM patients and healthy controls (HC), and its association with disease-specific features.

Methods: Thirty nine patients with IIM (32 females; mean age 56; mean disease duration 4. Read More

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Expanding the spectrum of the hyperferritinemic syndrome, from pathogenic mechanisms to clinical observations, and therapeutic implications.

Autoimmun Rev 2022 May 17:103114. Epub 2022 May 17.

Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.

From the introduction of hyperferritinemic syndrome concept, a growing body of evidence has suggested the role of ferritin as a pathogenic mediator and a relevant clinical feature in the management of patients with inflammatory diseases. From a pathogenic point of view, ferritin may directly stimulate the aberrant immune response by triggering the production of pro-inflammatory mediators in inducing a vicious pathogenic loop and contributing to the occurrence of cytokine storm syndrome. The latter has been recently defined as a clinical picture characterised by elevated circulating cytokine levels, acute systemic inflammatory symptoms, and secondary organ dysfunction beyond that which could be attributed to a normal response to a pathogen It is noteworthy that the occurrence of hyperferritinemia may be correlated with the development of the cytokine storm syndrome in the context of an inflammatory disease. Read More

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Autoantibodies and Cancer Association: the Case of Systemic Sclerosis and Dermatomyositis.

Clin Rev Allergy Immunol 2022 May 20. Epub 2022 May 20.

Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Several rheumatic diseases have a perplexing association with cancer. Unraveling this mysterious connection is likely to provide deeper understanding regarding mechanisms governing the onset of both autoimmunity and cancer immunity, in addition to providing clinicians much needed guidance around whom and when to screen for occult malignancy. Systemic sclerosis (scleroderma) and dermatomyositis are two diseases in which the association with internal malignancy is well-described and can be considered as models from which to gain important insights that likely have broader applicability. Read More

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Resistin Expression Is Associated With Interstitial Lung Disease in Dermatomyositis.

Front Med (Lausanne) 2022 3;9:903887. Epub 2022 May 3.

Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital, Beijing, China.

Objective: In the current study, we aimed to assess resistin mRNA levels in the peripheral blood mononuclear cells (PBMCs) of dermatomyositis patients with interstitial lung disease (DM-ILD) and their correlation with disease activity.

Methods: We detected resistin mRNA levels in the PBMCs of 37 DM-ILD, 8 DM patients without ILD, and 19 healthy control (HC) subjects by performing quantitative reverse transcription real-time polymerase chain reaction analysis. Associations between resistin expression levels and major clinical manifestations, laboratory examinations, and disease activity were also analyzed. Read More

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Not a Statin-Induced Myopathy: Metastatic Pancreatic Adenocarcinoma Presenting As Paraneoplastic Myositis.

Cureus 2022 May 15;14(5):e25016. Epub 2022 May 15.

Internal Medicine, Saint Francis Hospital, Hartford, USA.

Polymyositis is an inflammatory disease that causes bilateral proximal muscle weakness; unlike dermatomyositis, it is not usually associated with malignancy. However, there are a handful of case reports documenting polymyositis in patients with lymphoma, breast, lung, and bladder cancer. Here we report a case of metastatic pancreatic adenocarcinoma disguised by presenting as polymyositis. Read More

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[Treatment of Dermatomyositis and Immune-Mediated Necrotizing Myopathy with Poor Muscle Recovery with Steroids and IVIg: Therapeutic Strategies for Refractory Inflammatory Myositis].

Brain Nerve 2022 May;74(5):545-552

Department of Neurology, JA Toride Medical Center.

The majority of inflammatory myositis cases can be cured by immunomodulatory therapies. We recently observed that the phenotype and response to therapies differed according to myositis-specific autoantibodies; therefore, it is essential to select a suitable therapy after thoroughly evaluating the autoantibody, clinical severity, and complications. In some cases, the symptoms can be controlled by steroid monotherapy, but some cases exhibit steroid resistance and require other therapies. Read More

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[Treatment of Dermatomyositis and Immune-Mediated Necrotizing Myopathy with Poor Muscle Recovery with Steroids and IVIg: Intractable Dermatomyositis and Immune-Mediated Necrotizing Myopathy].

Kazuma Sugie

Brain Nerve 2022 May;74(5):537-544

Department of Neurology, Nara Medical University School of Medicine.

Among idiopathic inflammatory myopathies, dermatomyositis and immune-mediated necrotizing myopathy are distinguished by their different clinicopathological features. Corticosteroids are administered as the first-line treatment for both, and immunosuppressive agents and intravenous immunoglobulin important second-line treatments. Since some patients show resistance to these therapies, it is necessary to considering additional treatment based on muscle pathology, muscle imaging, and systemic complications such as malignancy and interstitial lung disease, in addition to the careful evaluation of muscle strength. Read More

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Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis complicated with macrophage activation syndrome.

Ther Adv Chronic Dis 2022 13;13:20406223221098128. Epub 2022 May 13.

Department of Rheumatology, Key Laboratory of Myositis, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, 100029 Beijing, China.

Background: Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM) has low survival rate, whereas macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. Their coexistence is very rare. This study aimed to describe the prevalence, clinical characteristics, and outcomes of anti-MDA5 antibodies-positive DM patients complicated with MAS. Read More

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Nailfold capillaroscopy for the early diagnosis of the scleroderma spectrum of diseases in patients without Raynaud's phenomenon.

J Scleroderma Relat Disord 2022 Jun 21;7(2):144-150. Epub 2022 Apr 21.

Department of Rheumatology and Immunology, Singapore General Hospital, Singapore.

Background: The utility of nailfold capillaroscopy in the evaluation of patients without Raynaud's phenomenon is unclear.

Objective: This study aims to compare the utility of nailfold capillaroscopy for the early diagnosis of the scleroderma-spectrum of diseases in patients who present with Raynaud's phenomenon, undifferentiated non-Raynaud's phenomenon features and positive systemic sclerosis-associated antibodies without scleroderma-spectrum of disease features.

Methods: Eligible patients were divided into three referral criteria groups: (I) Raynaud's phenomenon; (II) Undifferentiated non-Raynaud's phenomenon features and (III) Positive systemic sclerosis-associated autoantibodies without features to suggest scleroderma-spectrum of diseases. Read More

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Therapy-resistant dermatomyositis with extensive 'lumbar belt' calcinosis.

BMJ Case Rep 2022 May 18;15(5). Epub 2022 May 18.

Systemic Autoimmune and Rares Diseases Unit. Internal Medicine Department, Hospital Virgen del Rocio, Seville, Spain.

Calcinosis cutis (CC) is the umbrella term for calcium salt deposition on skin and subcutaneous tissue. We present a unique case of CC associated with anti-Mi2-positive dermatomyositis, having a distinctive distribution of subcutaneous calcifications appearing as a 'lumbar belt'. Treatment of CC remains challenging for clinicians due to a lack of high-quality evidence. Read More

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ELISA, protein immunoprecipitation and line blot assays for anti-TIF1-gamma autoantibody detection in cancer-associated dermatomyositis.

Rheumatology (Oxford) 2022 May 17. Epub 2022 May 17.

Division of Rheumatology, Karolinska University Hospital, Stockholm, Sweden.

Objectives: Anti-TIF1-gamma autoantibodies can be detected with immunoprecipitation (IP), line blot (LB), and enzyme-linked immunosorbent assay (ELISA). We compared assay performance in patients with dermatomyositis (DM) and the potential of these assays to detect anti-TIF1-gamma positive cancer-associated dermatomyositis (CADM).

Methods: We included sera from 131 patients with DM followed at Karolinska University Hospital, Stockholm, Sweden and 82 healthy controls. Read More

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Pain profile and opioid medication use in patients with idiopathic inflammatory myopathies.

Rheumatology (Oxford) 2022 May 17. Epub 2022 May 17.

Myositis Support and Understanding, Lincoln, DE.

Objectives: Pain is commonly reported in people living with myositis. This study assesses the presence of pain in the subtypes of myositis as well frequency of opioid and non-opioid pain medication use.

Methods: A survey was developed and distributed by Myositis Support and Understanding, a patient-led advocacy organization, to members of its group. Read More

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Adipose tissue distribution is associated with cardio-metabolic alterations in adult patients with juvenile-onset dermatomyositis.

Rheumatology (Oxford) 2022 May 16. Epub 2022 May 16.

Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Objectives: Primary aims were to compare adipose tissue distribution in adult patients with juvenile-onset dermatomyositis (JDM), with matched controls. Secondary aims were to explore how adipose tissue distribution is associated with cardio-metabolic status (cardiac dysfunction and metabolic syndrome) in patients.

Methods: Thirty-nine JDM patients (all aged ≥18 y, mean age 31. Read More

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Treatment of MDA5-positive dermatomyositis complicated by gangrenous cholecystitis with tofacitinib.

Eur J Med Res 2022 May 15;27(1):68. Epub 2022 May 15.

Suining Central Hospital, 127 Desheng West Road, Chuanshan, Suining, 629000, Sichuan, China.

Background: Dermatomyositis is a rare idiopathic inflammatory disease with diverse presentations that can have varying degrees of cutaneous and systemic involvement. This phenotypic heterogeneity makes DM a therapeutic challenge. Some therapeutic drugs, such as hormones and immunosuppressants, have poor therapeutic effects. Read More

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Management of calcinosis cutis in rheumatic diseases.

J Rheumatol 2022 May 15. Epub 2022 May 15.

Hadiya Elahmar MD, Dermatologist at U-turn Dermatology Clinic, Kuwait; Dermatology and Venerology, Ain Shams University, Cairo, Egypt. Brian M Feldman MD MSc, Pediatrics, Medicine, Institute of Health Policy Management and Evaluation, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. Sindhu R Johnson MD PhD, Toronto Scleroderma Program, Mount Sinai Hospital, Toronto Western Hospital, Institute of Health Policy Management and Evaluation, University of Toronto, Toronto, Ontario, Canada. Funding: Dr. Johnson is supported by a Canadian Institutes of Health Research New Investigator Award, Gurmej Kaur Dhanda Scleroderma Research Award, and the Oscar and Elanor Markovitz Scleroderma Research Fund. Dr. Feldman holds the Ho Family Chair in Autoimmune Diseases. Corresponding Author. Dr. Sindhu Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, Ontario, Canada, M5T 2S8. Phone 1-416-603-6417 Fax.1-416-603-4348. Email:

Calcinosis (hydroxyapatite and calcium phosphate crystal deposition) within the extracellular matrix of the dermis and subcutaneous tissue is a frequent manifestation of adult and pediatric systemic autoimmune rheumatic diseases, specifically systemic sclerosis, dermatomyositis, mixed connective tissue disease and systemic lupus erythematosus. In this article, we review classification of calcinosis, highlight mechanisms that may contribute to the pathogenesis of calcinosis and summarize the evidence evaluating non-pharmacologic and pharmacologic interventions for the treatment of calcinosis. Read More

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Evolving classification and role of muscle biopsy in diagnosis of inflammatory myopathies.

Indian J Pathol Microbiol 2022 May;65(Supplement):S241-S251

Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.

Idiopathic inflammatory myopathy (IIM) is a broad term that includes dermatomyositis, polymyositis, overlap myositis, sporadic inclusion body myositis, and immune-mediated necrotizing myopathy. The understanding of the pathogenesis of IIM is ever-evolving with regular updates in the classification schema. With the recognition of autoantibodies and their detection, the diagnostic algorithms are changing in favor of non-invasive diagnoses. Read More

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Development of dermatomyositis after anti-transcriptional intermediary factor 1-γ antibody seroconversion during treatment for small cell lung cancer.

BMC Pulm Med 2022 May 12;22(1):191. Epub 2022 May 12.

Department of Pulmonary Medicine, School of Medicine, Fukushima Medical University, 1 Hikarigaoka, Fukushima, 960-1295, Japan.

Background: Inflammatory myositis, such as dermatomyositis, is sometimes complicated by cancer and is recognized as cancer-associated myositis. Although some autoimmune antibodies are considered to be involved in the development of myositis in cancer patients, the precise mechanism has not been clarified. The findings of the present case shed light on the mechanism by which anti-transcriptional intermediary factor 1 (TIF1)-γ Ab was produced and the pathogenesis of cancer-associated myositis. Read More

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