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Cureus 2023 Aug 21;15(8):e43887. Epub 2023 Aug 21.

Internal Medicine, Saint Vincent Hospital, Worcester, USA.

Polymyositis is an autoimmune multisystemic disorder that affects the body's muscular system. It usually affects the proximal muscles of the shoulder, pelvis, neck flexor muscles, and sometimes, the hip extensor muscles. However, it can also affect the diaphragm causing acute respiratory failure. Read More

Pediatr Rheumatol Online J 2023 Sep 21;21(1):106. Epub 2023 Sep 21.

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

Background: Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. The coexistence of MAS and juvenile dermatomyositis (JDM) is not well reported. This report describes a case of JDM with MAS and summarizes the clinical characteristics and prognosis of MAS in patients with JDM. Read More

Front Immunol 2023 5;14:1177721. Epub 2023 Sep 5.

Department of Neurology, University Medical Center Göttingen, Göttingen, Germany.

Introduction: We aimed to identify B-cell-mediated immunomechanisms in inclusion body myositis (IBM) and polymyositis (PM) as part of the complex pathophysiology.

Materials And Methods: Human primary myotube cultures were derived from orthopedic surgery. Diagnostic biopsy specimens from patients with IBM (n=9) and PM (n=9) were analyzed for markers of B cell activation (BAFF and APRIL) and for chemokines that control the recruitment of B cells (CXCL-12 and CXCL-13). Read More

J Int Med Res 2023 Sep;51(9):3000605231198742

Department of Rheumatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, China.

The prognosis of anti-MDA5-positive dermatomyositis (DM)-associated rapidly progressive interstitial lung disease (RPILD) is extremely poor and effective treatment options are limited. In addition, the risk of infection during immunosuppressive treatment is a major challenge. We report here, a case of RPILD in a 31-year-old man with anti-MDA5 antibody-positive DM. Read More

ARP Rheumatol 2023 May 30. Epub 2023 May 30.

Hospital CUF Cascais.

Aims: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort.

Methods: Reuma. Read More

Pediatr Rheumatol Online J 2023 Sep 19;21(1):104. Epub 2023 Sep 19.

Faculty of Medicine, Department of Pediatrics, Pediatric Rheumatology and Immunology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

Background: Juvenile dermatomyositis (jDM) is the most common idiopathic inflammatory myopathy of childhood. Amyopathic or hypomyopathic courses have been described.

Case Presentation: We present the case of a 4-year-old patient with MDA5 antibody positive jDM and interstitial lung disease. Read More

Ann Clin Transl Neurol 2023 Sep 18. Epub 2023 Sep 18.

Department of Neurology, Peking University First Hospital, Beijing, 100034, China.

Objective: Anti-mitochondrial antibodies (AMAs) are associated with idiopathic inflammatory myopathies (IIMs). We aimed to summarize the clinicopathological characteristics, assess circulating cell-free mitochondrial DNA (ccf-mtDNA), and circulating cell-free nuclear DNA (ccf-nDNA) in AMA-associated IIMs.

Methods: Medical records of 37 IIMs patients with AMAs were reviewed. Read More

Cureus 2023 Aug 16;15(8):e43584. Epub 2023 Aug 16.

Surgery, Dow University of Health Sciences, Karachi, PAK.

We describe a rare case of concurrent eosinophilic granulomatosis with polyangiitis and mixed connective tissue disease in a 27-year-old man who presented with pulmonary, renal, cardiac, and skin manifestations. We confirmed the diagnosis based on clinical, histopathological, and serological criteria. We treated the patient with corticosteroids, methotrexate, cyclophosphamide, and hydroxychloroquine, achieving early remission. Read More

Indian J Crit Care Med 2023 Sep;27(9):690-691

Department of Intensive Care and Medicine, Gurugram, Haryana, India.

: Bansal T. Is It Polymyositis? Indian J Crit Care Med 2023;27(9):690-691. Read More

Autoimmun Rev 2023 Sep 14;22(11):103447. Epub 2023 Sep 14.

Department of Clinical Internal, Anesthesiological and Cardiovascular Sciences, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy. Electronic address:

Autoimmune diseases have specific pathophysiologic mechanisms leading to an increased risk of arterial and venous thrombosis. The risk of venous thromboembolism (VTE) varies according to the type and stage of the disease, and to concomitant treatments. In this review, we revise the most common autoimmune disease such as antiphospholipid syndrome, inflammatory myositis, polymyositis and dermatomyositis, rheumatoid arthritis, sarcoidosis, Sjogren syndrome, autoimmune haemolytic anaemia, systemic lupus erythematosus, systemic sclerosis, vasculitis and inflammatory bowel disease. Read More

J Coll Physicians Surg Pak 2023 Jan;33(1):9-11

Department of Medicine and Allied, Azra Naheed Medical College, Superior University, Lahore, Pakistan.

Mixed Connective Tissue Disease (MCTD) is an overlap syndrome of mild severity and good outcome. This disease consists of clinical features overlapping between Systemic Lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, and polymyositis, in the presence of specific anti-RNP antibodies. We report a case of a 20-year girl who presented with a 3-month history of joint pains involving the small joints of her hands along with morning stiffness, skin thickening of hands, Raynauds phenomena, and recurrent photosensitive skin rashes. Read More

Clin Exp Rheumatol 2023 Sep 14. Epub 2023 Sep 14.

Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.

Objectives: Patients with systemic vasculitis may develop myalgia as an initial symptom. However, the immunopathology of vasculitic myopathy remains unclear. We investigated the immunopathological features of skeletal muscle in small-to-medium-sized vessel vasculitis. Read More

Front Immunol 2023 28;14:1249844. Epub 2023 Aug 28.

Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

Objective: This study aimed to investigate the relationship between anti-MDA5 titer and type I IFN signature in patients with MDA5+ DM.

Methods: We explored the transcriptome profiling of PBMCs in MDA5+ DM patients with high-titer of antibody at disease onset or relapse and normal low-titer after treatment and healthy donors. Subsequently, we revealed the dynamic relationship between serum type I IFN scores and antibody titers. Read More

Front Immunol 2023 28;14:1255540. Epub 2023 Aug 28.

Department of Dermatology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.

Comprehensive autoantibody evaluation is essential for the management of autoimmune disorders. However, conventional methods suffer from poor sensitivity, low throughput, or limited availability. Here, using a proteome-wide human cDNA library, we developed a novel multiplex protein assay (autoantibody array assay; A-Cube) covering 65 antigens of 43 autoantibodies that are associated with systemic sclerosis (SSc) and polymyositis/dermatomyositis (PM/DM). Read More

Cureus 2023 Aug 11;15(8):e43337. Epub 2023 Aug 11.

Neurology, Hospital General de México Dr. Eduardo Liceaga, Mexico City, MEX.

Inflammatory myopathies are a group of diseases whose common pathway is immune-mediated muscle damage, one of which is polymyositis. The definition of polymyositis is controversial, with proponents advocating a definition based on immunohistochemical and histopathological findings in muscle biopsies, while other proponents advocate a definition based on clinical manifestations and histopathological findings. Polymyositis is a quite rare disease that is clinically characterized by progressive proximal muscle weakness with a symmetric distribution. Read More

N Engl J Med 2023 Sep 9;389(11):1032. Epub 2023 Sep 9.

Kaohsiung Medical University, Kaohsiung, Taiwan

Front Immunol 2023 24;14:1209282. Epub 2023 Aug 24.

Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.

Background: Rapidly progressive interstitial lung disease (RP-ILD) is the most serious complication of anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5 DM). This study was performed to assess the prognostic factors of patients with anti-MDA5 DM and the clinical characteristics and predictors of anti-MDA5 DM in combination with RP-ILD.

Methods: In total, 73 MDA5 DM patients were enrolled in this study from March 2017 to December 2021. Read More

Skeletal Radiol 2023 Sep 6. Epub 2023 Sep 6.

Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, Republic of Korea.

Inflammatory myopathies, such as polymyositis and dermatomyositis, are systemic inflammatory disorders that affect skeletal muscles and internal organs. The treatment of inflammatory myopathies usually involves long-term use of high doses of steroids and/or immunosuppressants, making patients susceptible to opportunistic infections. Unfortunately, infections are a leading cause of morbidity and mortality in patients with inflammatory myopathies. Read More

RMD Open 2023 Sep;9(3)

Department of neurology, Neurological Institute Mondino, University of Pavia, Pavia, Italy.

Background: Some myopathies can lead to dropped head or bent spine syndrome (DH/BS). The significance of this symptom has not been studied in inflammatory myopathies (IM).

Objectives: To assess the significance of DH/BS in patients with IM. Read More

Orphanet J Rare Dis 2023 Aug 31;18(1):256. Epub 2023 Aug 31.

Internal Medicine Department, Hospital Clínic, University of Barcelona and CIBERER (Madrid), C/Villarroel 170, 08036, Barcelona, Spain.

Background: The screening of high-risk populations using dried blood spots (DBS) has allowed the rapid identification of patients with Pompe disease, mostly in Neurology departments. The aim of the study was to determine the prevalence of late-onset Pompe disease (LOPD) among patients not previously diagnosed or tested for this entity despite presenting possible signs or symptoms of the disease in Internal Medicine departments in Spain.

Methods: This epidemiological, observational, cross-sectional, multicenter study included a single cohort of individuals with clinical suspicion of LOPD seen at Internal Medicine departments in Spain. Read More

RMD Open 2023 Aug;9(3)

Research and Development, Janssen Pharmaceutical KK, Tokyo, Japan.

Objectives: To evaluate the efficacy and safety of ustekinumab (UST) in a multicentre, randomised, double-blind, placebo-controlled trial in adult Japanese patients with active polymyositis (PM) and dermatomyositis (DM).

Methods: Fifty-one Japanese adults diagnosed with active PM/DM who did not respond adequately to one or more standard-of-care treatments were randomised 1:1 to receive UST (n=25) or placebo (n=26). Participants received body weight-range based intravenous administration of UST (6 mg/kg) or placebo at week 0 followed by 90 mg subcutaneous (SC) administration of UST or placebo every 8 weeks from week 8 to week 24. Read More

RMD Open 2023 Aug;9(3)

Division of Rheumatology, Department of Internal Medicine, The Jikei University School of Medicine, Tokyo, Japan.

Objectives: Anti-differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis, which has been described as clinically amyopathic dermatomyositis, complicates rapidly progressive interstitial lung disease (ILD). Owing to the absence of significant muscle symptoms, musculoskeletal MRI is often not performed. In this study, we aimed to devise a simple evaluation method using musculoskeletal MRI findings to elucidate the relationship between MRI findings and ILD prognosis and development. Read More

Muscle Nerve 2023 Aug 28. Epub 2023 Aug 28.

Department of Neuromuscular Medicine, Hospital for Special Care, New Britain, Connecticut, USA.

Introduction/aims: In our experience, patients with late-onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) are frequently misdiagnosed, some for many years. The aim of this report is to document this clinical experience including the presenting symptoms and misdiagnoses and to discuss the challenges in diagnosing patients with late-onset FSHD1.

Methods: We performed a retrospective medical record review and recorded clinical data on patients with a genetically confirmed diagnosis of FSHD1, who began to have symptoms at 50 years of age or older, and either had no family history of FSHD1 or had a history of an undiagnosed weakness in a family member. Read More

Cureus 2023 Jul 27;15(7):e42575. Epub 2023 Jul 27.

Sleep Medicine, Staten Island University Hospital, Staten Island, USA.

This case report describes an 82-year-old female patient with myasthenia gravis (MG) who presented with worsening dysphagia. The patient was found to have cricopharyngeal sphincter and esophageal achalasia, and a percutaneous endoscopic gastrostomy (PEG) tube was placed due to severe pharyngeal dysphagia and cricopharyngeal dysfunction. The patient had class IVb myasthenia gravis and was treated with intravenous immunoglobulin (IVIG), prednisone, and pyridostigmine. Read More

Neurol Sci 2023 Aug 25. Epub 2023 Aug 25.

Department of Neuroscience, Mental Health and Sensory Organs (NESMOS), Faculty of Medicine and Psychology, Neuromuscular and Rare Disease Centre, Sant'Andrea Hospital, SAPIENZA University of Rome, Via Di Grottarossa 1035-1039, 00189, Rome, Italy.

Introduction: Mitochondrial alterations are a common finding in muscle biopsy of sporadic inclusion body myositis (s-IBM) and polymyositis with mitochondrial pathology (PM-Mito). Both disorders generally have poor treatment response. Nevertheless, mitochondrial myopathology has been rarely reported in dermatomyositis (DM) outside areas of perifascicular atrophy and a relationship with therapeutic outcome is not established. Read More

Appl Biochem Biotechnol 2023 Aug 25. Epub 2023 Aug 25.

Department of Neurology, Affiliated Zhongda Hospital, Neuropsychiatric Institute, School of Medicine, Southeast University, No. 87, Dingjiaqiao, Gulou District, Nanjing, 210003, Jiangsu, China.

Polymyositis (PM) is the most common autoimmune disease in neurology and among muscle disorders; it is of great significance to thoroughly understand the mechanism of PM to find new diagnosis and treatment methods. This research intends to elucidate the clinical implications and mechanisms of complement C1q in polymyositis (PM). One hundred fifteen PM patients (research group, RG) and 120 healthy subjects (control group, CG) who visited our hospital between March 2017 and March 2020 were selected. Read More

Ugeskr Laeger 2023 Aug;185(33)

Klinik for Led- og Bindevævssygdomme, 4242, Afdeling for Rygkirurgi, Led- og Bindevævssygdomme, Københavns Universitetshospital - Rigshospitalet.

Cureus 2023 Jul 23;15(7):e42336. Epub 2023 Jul 23.

Internal Medicine, Florida Atlantic University Charles E. Schmidt College of Medicine, Boca Raton, USA.

Ulcerative colitis (UC) is an inflammatory bowel disease (IBD) that is thoroughly studied and known to have a strong genetic component. It affects the mucosa and submucosa of the colon and rectum, causing diffuse friability and superficial erosions leading to bleeding. Common presenting symptoms include diarrhea that is often bloody or purulent and abdominal pain or cramping. Read More

Autoimmun Rev 2023 Aug 22;22(10):103416. Epub 2023 Aug 22.

Rheumatology Unit, ERN-ReCONNET center, Meyer Children Hospital IRCCS, Florence, Italy; NEUROFARBA Department, University of Florence, Firenze, Italy.

Objective: Juvenile idiopathic inflammatory myopathies (JIIM) are a group of connective tissue disorders characterized by muscle inflammation and variable systemic involvement, including interstitial lung disease (ILD). Available data on JIIM-associated ILD are very limited. We performed a systematic review of the available clinical, laboratory, and radiological features of JIIM-associated ILD. Read More

J Clin Neuromuscul Dis 2023 Sep;25(1):46-50

Child Neurology Department, Hedi Chaker Sfax University Hospital, Sfax, Tunisia.

We report a 5-year-old boy who presented with progressive weakness in 4 limbs and gait disorders over 7 months. No skin rash was observed on admission. A symmetrical proximodistal weakness was found. Read More