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Cureus 2021 Jun 4;13(6):e15445. Epub 2021 Jun 4.

Rheumatology, Adventist Medical Center, Hanford, USA.

A 56-year-old Hispanic female presented with six weeks of progressive dysphagia, proximal muscle weakness, erythematous rash, bilateral upper extremity pitting edema, and left lower extremity pitting edema. She had preserved heart function and a normal echocardiogram (ECG). She presented with elevated creatine kinase (CK) and aldolase, with normal renal function. Read More

BMJ Case Rep 2021 Jun 9;14(6). Epub 2021 Jun 9.

Neurology, Hospital Universitari Son Espases, Palma de Mallorca, Balearic Islands, Spain.

Dropped head syndrome (DHS) is an uncommon clinical syndrome, which requires complex diagnostic evaluation. A variety of neuromuscular and neurodegenerative disease can produce weakness of head extensor muscles and consequently lead to head drop. Inflammatory myopathy has been described as a cause of DHS, however head drop has only exceptionally been reported as being the presenting symptom of this disorder. Read More

Case Rep Infect Dis 2021 20;2021:9998415. Epub 2021 May 20.

Section of Infectious Diseases, Department of Medicine, Yale School of Medicine, New Haven, CT 06520, USA.

Neuromuscular complications are common in patients with HIV/AIDS at any stage of the disease process. Myopathies can be secondary to antiretroviral therapy, HIV myositis itself, or other etiologies. Here, we present the case of a middle-aged male with HIV who presented with myalgias and was diagnosed with myotonic dystrophy and HIV-associated polymyositis after extensive workup including clinical history and physical exam, laboratory markers, electromyogram, and muscle biopsy. Read More

J Intern Med 2021 Jun 3. Epub 2021 Jun 3.

From the, Department of Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.

Background: Mounting evidence suggests that dermatomyositis/polymyositis (DM/PM) are associated with increased risk of atherosclerotic events and venous thromboembolism. However, data on the association between DM/PM and other cardiac outcomes, especially heart failure (HF), are scarce.

Objectives: To examine the long-term risk and prognosis associated with adverse cardiac outcomes in patients with DM/PM. Read More

Dermatology 2021 Jun 1:1-9. Epub 2021 Jun 1.

Department of Dermatology, Taichung Veterans General Hospital, Taichung, Taiwan.

Background: Up to 25% of patients with cutaneous lupus erythematosus (CLE) can develop systemic lupus erythematosus (SLE). However, the risk of autoimmune diseases other than SLE in CLE patients who have only skin manifestations (CLE-alone) has rarely been explored.

Objective: To investigate the long-term risk and independent factors of non-SLE autoimmune diseases among CLE-alone patients. Read More

Med Clin North Am 2021 Jul;105(4):757-782

Autoimmune Skin Disease Program, Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Connective tissue diseases (CTDs) encompass a broad spectrum of clinical presentations that involve multidisciplinary management. Cutaneous findings are common in CTD and careful examination of these features aids in appropriate diagnosis and subsequent evaluation. Thorough work-up of CTD is crucial to properly identify disease subtypes and systemic involvement. Read More

Cureus 2021 Apr 24;13(4):e14670. Epub 2021 Apr 24.

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

Introduction Approximately 5-10% of strokes occur in adults of less than 45 years of age. The rising prevalence of stroke risk factors may increase stroke rates in young adults (YA). We aimed to compare risk factors and outcomes of acute ischemic stroke (AIS) among YA. Read More

Intern Med 2021 May 29. Epub 2021 May 29.

Department of Nephrology, Toranomon Hospital, Japan.

We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. Read More

Heart Rhythm 2021 May 25. Epub 2021 May 25.

University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160.

Background: Systemic inflammation has been associated with atherosclerotic cardiovascular diseases (ASCVD) and arrhythmia occurrence in rheumatologic conditions such as rheumatoid arthritis. Polymyositis-Dermatomyositis (PD) are rare rheumatologic conditions characterized by symmetric proximal muscle weakness and cutaneous eruption in the case of dermatomyositis. Although, there is literature associating PD with ASCVD, there are no population level studies analyzing arrhythmia risk in PD. Read More

Ultrasound Med Biol 2021 May 21. Epub 2021 May 21.

Department of Diagnostic and Interventional Radiology, University Hospital Tübingen, Tübingen, Germany.

Changes in muscle elasticity are expected in patients with untreated myositis. The purpose of this study was to define the accuracy of shear-wave elastography (SWE) in diagnosing myositis. This case control study included 21 patients (mean age, 49. Read More

Int J Rheum Dis 2021 Jun 24;24(6):815-827. Epub 2021 May 24.

Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Aim: To investigate the risk factors for interstitial lung disease (ILD) and prognosis in patients with idiopathic inflammatory myopathy (IIM).

Methods: A retrospective longitudinal study was performed in patients diagnosed with IIM between January 2012 and December 2018.

Results: The study cohort included 91 men and 195 women who were classified as having dermatomyositis (DM, n = 183), polymyositis (PM, n = 77), or clinical amyopathic DM (CADM, n = 26). Read More

Semin Musculoskelet Radiol 2021 Feb 21;25(1):82-93. Epub 2021 May 21.

Department of Radiology and Medical Imaging, Ghent University Hospital, Ghent, Belgium.

This article reviews the application of magnetic resonance imaging (MRI) to pediatric rheumatic diseases. MRI can detect early manifestations of arthritis, evaluate the extent of disease, and monitor disease activity and response to treatment.Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disorder, representing a diverse group of related diseases that share a definition of joint inflammation of unknown origin with onset before 16 years of age and lasting > 6 weeks. Read More

Semin Musculoskelet Radiol 2021 Feb 21;25(1):68-81. Epub 2021 May 21.

Department of Radiology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.

Juvenile idiopathic arthritis is the most frequent rheumatic disease in the pediatric population, followed by systemic lupus erythematosus, juvenile scleroderma syndromes, juvenile dermatomyositis, chronic recurrent multifocal osteomyelitis, and juvenile vasculopathies. The imaging approach to inflammatory connective tissue diseases in childhood has not changed dramatically over the last decade, with radiographs still the leading method for bony pathology assessment, disease monitoring, and evaluation of growth disturbances. Ultrasonography is commonly used for early detection of alterations within the intra- and periarticular soft tissues, assessing their advancement and also disease monitoring. Read More

Cureus 2021 Apr 15;13(4):e14507. Epub 2021 Apr 15.

Neurology, Strong Memorial Hospital, Rochester, USA.

Idiopathic inflammatory myopathy encompasses a group of acquired, heterogeneous, systemic diseases of the skeletal muscle, including adult polymyositis, adult dermatomyositis, juvenile dermatomyositis, juvenile polymyositis, inclusion body myositis, and necrotizing myopathy, all resulting in muscle weakness. Granulomatous myositis (GM) is a rare myopathy disorder histologically characterized by the development of endomyseal and/or perimyseal granulomas in striated muscle. GM is often associated with sarcoidosis. Read More

Dermatol Ther 2021 May 14:e14976. Epub 2021 May 14.

Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China.

Anti-TNF treatment may be useful for patients with idiopathic inflammatory myopathies (IIMs). The purpose of this study is to assess the efficacy of infliximab (IFX) in the management of IIMs. Two databases (ie, PubMed and China National Knowledge Infrastructure) were searched up to Nov 2020 for studies investigating skin lesions and muscular weakness in patients with IIMs treated with IFX. Read More

Reumatologia 2021 27;59(2):115-120. Epub 2021 Apr 27.

Department of Systemic Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland.

Mixed connective tissue disease (MCTD) is a complex entity, which incorporates features of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc) and polymyositis/dermatomyositis (PM/DM). Nailfold videocapillaroscopy (NVC) is a simple, safe and non-invasive technique of capillary vessel assessment, allowing for qualitative and quantitative assessment of microcirculation. NVC plays a pivotal role in the diagnostic algorithm of connective tissue diseases, especially in systemic sclerosis (SSc). Read More

Rheumatology (Oxford) 2021 May 11. Epub 2021 May 11.

Service de Physiologie, Explorations Fonctionnelles Musculaires, CHU de Strasbourg, Strasbourg, France.

Objective: We recently recorded a high prevalence of inclusion body myositis (IBM) in patients with Sjögren's syndrome (SS). Whether myositis patients with SS differ from myositis patients without SS in terms of the characteristics of the myositis is currently unknown. Anti-cytosolic 5'-nucleotidase 1 A (cN1A) has recently been proposed as a biomarker for IBM but is also frequent in SS. Read More

Arthritis Care Res (Hoboken) 2021 May 10. Epub 2021 May 10.

Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan.

Objectives: The aim of this study was to determine the prognosis, clinical course, and current management of severe interstitial lung disease (ILD) associated with myositis in Japan.

Methods: We conducted a retrospective descriptive study using a nation-wide database for inpatient care of acute illness in Japan. Among a total of approximately 66 million inpatient admissions, we identified patients with severe ILD associated with polymyositis (PM) or dermatomyositis (DM) which required mechanical ventilation and methylprednisolone pulse therapy (≥1g/day usage of methylprednisolone) from July 2010 to March 2018. Read More

Sci Rep 2021 May 10;11(1):9821. Epub 2021 May 10.

Department of Radiology, Saitama Medical University Hospital, 38 Morohongo Moroyama-machi, Iruma-gun, Saitama, Japan.

To develop a machine learning (ML) model that predicts disease groups or autoantibodies in patients with idiopathic inflammatory myopathies (IIMs) using muscle MRI radiomics features. Twenty-two patients with dermatomyositis (DM), 14 with amyopathic dermatomyositis (ADM), 19 with polymyositis (PM) and 19 with non-IIM were enrolled. Using 2D manual segmentation, 93 original features as well as 93 local binary pattern (LBP) features were extracted from MRI (short-tau inversion recovery [STIR] imaging) of proximal limb muscles. Read More

Clin Dermatol 2021 Jan-Feb;39(1):56-63. Epub 2020 Dec 15.

Department of Dermatology and Venereology, Medical University, Sofia, Bulgaria.

Autoimmune connective tissue diseases are a heterogeneous group of clinical entities sharing a common feature-an impairment of structural components like collagen and elastin, arising by autoimmune mechanisms. Because most patients are on a long-term immunosuppressive therapy, which renders them vulnerable to infections, a new challenge appears in front of physicians in the coronavirus disease 2019 (COVID-19) era. Immune mechanisms are substantial for the control and ceasing of viral infections, and their impairment may cause serious complications; however, data from immunosuppressed transplant patients do not reveal a higher frequency or diseases' severity in those infected by COVID-19. Read More

Autoimmun Rev 2021 Jul 7;20(7):102851. Epub 2021 May 7.

Division of Rheumatology, Centre hospitalier de l'Université de Montréal (CHUM), CHUM Research Center, Department of Medicine, Université de Montréal, Montréal, Québec, Canada. Electronic address:

Background: Scleromyositis (SM) is an emerging subset of myositis associated with features of systemic sclerosis (SSc) but it is currently not recognized as a distinct histopathological subset by the European NeuroMuscular Center (ENMC). Our aim was to review studies reporting muscle biopsies from SSc patients with myositis and to identify unique histopathological features of SM.

Methods: A scoping review was conducted and included all studies reporting histopathological findings in SSc patients with myositis searching the following databases: PubMed, MEDLINE, EMBASE, CINAHL and EBM-Reviews. Read More

BMC Pulm Med 2021 May 5;21(1):150. Epub 2021 May 5.

Department of Intensive Care Medicine, Northern Hospital, Melbourne, Australia.

Introduction: Acute exacerbation (AE) is a devastating phenomenon and reported to be complicated with systemic autoimmune disease-associated interstitial lung disease (ILD). The aim of this study was to investigate the incidence and prognosis of AE of systemic autoimmune disease-ILD and clarify relevant clinical information predictive of these outcomes.

Method: This study was designed as a systematic review and meta-analysis. Read More

J Clin Rheumatol 2021 Apr 28. Epub 2021 Apr 28.

From the Department of Autoimmune Diseases, Institut Clinic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Catalonia, Spain Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Sabadell, Barcelona, Catalonia Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Vizcaya Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza Department of Internal Medicine, Hospital Campus de la Salud, Complejo Universitario de Granada Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Barcelona Department of Internal Medicine, Hospital Clínico Universitario de Santiago, Santiago de Compostela, A Coruña Department of Internal Medicine, Hospital La Paz, Madrid Department of Internal Medicine, Hospital Universitari Mutua de Terrassa, Terrassa, Barcelona, Catalonia Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain.

Objectives: To assess the clinical profile of patients with anti-polymyositis/Scl (PM/Scl) antibodies in a cohort of Spanish patients with systemic sclerosis.

Methods: From the Spanish Scleroderma Study Group database, we selected patients in whom PM/Scl antibodies had been tested. We compared demographic, clinical, laboratory, and survival data between patients with and without PM/Scl antibodies. Read More

Rheumatology (Oxford) 2021 Apr 30. Epub 2021 Apr 30.

Division of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Chongqing, China.

Int J Environ Res Public Health 2021 04 10;18(8). Epub 2021 Apr 10.

Laboratory for Industrial and Applied Mathematics (LIAM), Department of Mathematics and Statistics, York University, Toronto, ON M3J 1P3, Canada.

Polymyositis (PM) and dermatomyositis (DM) are autoimmune-mediated multisystemic myopathies, characterized mainly by proximal muscle weakness. A connection between epilepsy and PM/DM has not been reported previously. Our study aim is to evaluate this association. Read More

Medicina (Kaunas) 2021 Apr 3;57(4). Epub 2021 Apr 3.

Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu 431-3192, Japan.

Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. The management of PM-/DM-/CADM-associated ILD (PM/DM/CADM-ILD) requires careful evaluation of the disease severity and clinical subtype, including the ILD forms (acute/subacute or chronic), because of the substantial heterogeneity of their clinical courses. Read More

Ann Med Surg (Lond) 2021 May 4;65:102269. Epub 2021 Apr 4.

1st Department of Surgery and Interventional Gastroenterology, Semmelweis University, Budapest, 1085, Budapest, Üllői út 78, Hungary.

Introduction: Rhabdomyolysis is a syndrome characterized by a rapid necrosis of muscle fibers and the release of muscle-derived metabolic products into the circulatory system. A rare cause of rhabdomyolysis is paraneoplastic polymyositis.

Case Presentation: A 67-year-old man was diagnosed with paraneoplastic polymyositis and rhabdomyolysis caused by hepatocellular carcinoma (HCC). Read More

BMJ Case Rep 2021 Apr 28;14(4). Epub 2021 Apr 28.

Department of Respiratory Medicine, Amrita Institute of Medical Science, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India.

Melanoma differentiation-associated protein 5 (MDA5) antibody-positive dermatomyositis (DM) displays unique cutaneous and pathologic features. We describe two cases of myositis-associated rapidly progressive interstitial lung disease (RP-ILD). The patients were two women from Kerala, India. Read More

Yonsei Med J 2021 May;62(5):424-430

Division of Rheumatology, Department of Internal Medicine, Kyung Hee University Medical Center, Seoul, Korea.

Purpose: To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients.

Materials And Methods: We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Read More

Am J Case Rep 2021 Apr 27;22:e930286. Epub 2021 Apr 27.

Department of Respiratory Medicine, Hamanomachi Hospital, Fukuoka City, Fukuoka, Japan.

BACKGROUND Pembrolizumab is a humanized monoclonal antibody against programmed cell death-1 protein. Pembrolizumab sometimes causes immune-related adverse events (irAEs). Dermatomyositis is a rare irAE of immune checkpoint inhibitors. Read More