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Clinical Value of Bronchiectasis and Airspace Enlargement Depicted on Iodine Map in Patients With Interstitial Lung Disease: A Pilot Study.

Objectives To investigate the degree and clinical meaning of bronchiectasis and airspace enlargement depicted on the iodine map in dual-ener … s by most readers. On the contrary, image finding was ameliorated in those regions in patients with dermatomyositis. Conclusions Bronchiectasis and airspace enlargement in the iodine map are depicted more prominently compared to the monochromatic image in dual-energy CT pulmonary angiography and have various time courses based on interstitial lung disease subtype …

Small cell lung cancer with peripheral neuropathy as the first symptom: Two case reports.

BACKGROUND: Small cell lung cancer (SCLC) is the most malignant type of lung cancer. Even in the latent period and early stage of the tumor, … lly aggravated. The patient was later diagnosed with SCLC, and the dyskinesia did not worsen as the dermatomyositis improved after antineoplastic and hormone therapy. The second case presented with limb numbness and weakness as the first symptom, but the patient did not pay attention to it. Later, the patient was diagnosed with SCLC after facial edema caused by t …

Fragmentation signatures in cancer patients resemble those of patients with vascular or autoimmune diseases.

Multiple case-controlled studies have shown that analyzing fragmentation patterns in plasma cell-free DNA (cfDNA) can distinguish individual … tifs in cfDNA. We found that individuals with venous thromboembolism, systemic lupus erythematosus, dermatomyositis, or scleroderma have cfDNA fragmentation signatures that closely resemble those found in individuals with advanced cancers. Furthermore, these signatures were highly correlated with increases in inflammatory markers in the blood. We demonstrate that …

Type I interferon signature: a quantitative standardized method for clinical application.

Type I Interferon (IFN) induced gene expression analysis ("IFN signature") is employed to categorize pathological conditions that exhibit Ty … ted or not to Type I IFN (i.e. monogenic interferonopathies, systemic lupus erythematosus, juvenile dermatomyositis, periodic fevers, and juvenile idiopathic arthritis). The proposed method proved to be highly reproducible among centers and able to discriminate among IFN-related or non-IFN-related inflammation. The use of a synthetic control minimized the inter-a …

The Impact of Tobacco Smoking on Systemic Sclerosis, Idiopathic Inflammatory Myositis, and Systemic Lupus Erythematosus.

This narrative review aims specifically to explore the relationship between tobacco exposure and systemic sclerosis (SSc), idiopathic inflam … garette smoking has been associated with IIM through genetic and molecular mechanisms. Smokers with dermatomyositis or polymyositis are at an elevated risk of atherosclerosis and interstitial lung disease. Similarly, smoking in patients with SLE increases the risk of organ damage, thrombosis, and disease severity compared with non-smokers. Smokers with SLE also h …

Клеточная терапия ревматических и мышечных заболеваний.

Значительная часть пациентов, у которых диагностированы ревматологические и мускулоскелетные заболевания (РМ), демонстрируют устойчивость к традиционной терапии или испытывают повторяющиеся симптомы. Эти заболевания, к которым относятся аутоиммунные расстройства, такие, как множественный склероз, ревматоидный артрит и системный волшебный эритематос, характеризуются наличием автоматически реактивных клеток B, играющих решающую роль в их патогенезе. Стойкость этих самореактивных клеток В в лимфатических органах и воспаленных тканях снижает эффективность таких моноклонных антител, как ритуксимаб,

Совпадение в аннотации оригинала
A substantial proportion of patients diagnosed with rheumatologic and musculoskeletal diseases (RMDs) exhibit resistance to conventional the … B cells. Preliminary data from patients with RMDs, particularly those with lupus erythematosus and dermatomyositis, suggest that CAR T-cells targeting CD19 can induce rapid and sustained depletion of circulating B cells, leading to complete clinical and serological responses in cases that were previously unresponsive to conventional therapies. This review will p …

A Stepwise Progression of Acute Bilateral Visual Loss Due to Onodi Cell Sinusitis.

Acute visual loss in an immunocompromised patient may be caused by acute invasive fungal sinusitis (AIFS), even if symptoms include only mild headache and computed tomography (CT) shows only mild sinusitis, especially of the Onodi cell. Herein, we report a case of a 71-year-old man with a medical history of dermatomyositis and type 2 diabetes mellitus who presented with a stepwise progression of acute bilateral visual loss, mild headache, and altered consciousness. Initially, as the plain cranial CT showed only mild fluid retention in the posterior ethmoid sinus without bone destruction, the s…

Osteopoikilosis: a case report.

BACKGROUND: Osteopoikilosis, also referred to as disseminated condensing osteopathy, spotted bone disease, or osteopecilia, is a rare bone disorder. The case presented here showcases multiple disc lesions in the spine, extensive multifocal skin lesions, and positive test results for dermatomyositis and multifocal enthesopathy, accompanied by neurological symptoms. This manifestation represents a novel variant of the disease. CASE PRESENTATION: Our patient is a 46-year-old mosque Kurdish servant presenting with complaints of pain in the right leg, lower back, right hand, and neck. Additionally…

The role of trichoscopy beyond hair and scalp diseases. A review.

Trichoscopy is a diagnostic tool for hair and scalp diseases. It was recently shown that it also allows the identification of features associated with disorders that typically do not affect the scalp. The aim of this article was to analyse and outline the usefulness of trichoscopy in suspecting such diseases. Connective tissue diseases were the most investigated systemic disorders in regard to trichoscopy. The most common features of systemic lupus erythematosus, systemic sclerosis and dermatomyositis are thick arborizing and tortuous vessels. Avascular areas are present in systemic sclerosis.…

LINE-1: an emerging initiator of cGAS-STING signalling and inflammation that is dysregulated in disease.

The cGAS-STING (cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING)) axis integrates DNA damage and cellular stress with t … f type I IFN signalling through cGAS-STING, such as Aicardi-Goutières syndrome, Fanconi anemia, and dermatomyositis. Although TREX1 is highly conserved in tetrapod species, other suppressor proteins exist that inhibit L1 retrotransposition. These suppressor genes when mutated are often associated with diseases characterized by unchecked inflammation that is assoc …

Improving Vitamin D Screening in a Pediatric Rheumatology Clinic Using Structured Quality Improvement Process.

INTRODUCTION: Monitoring levels of 25-hydroxyvitamin D (25-OHD) is an integral part of bone health assessment in the general pediatric population, especially in at-risk populations such as children with juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (c-SLE), and juvenile dermatomyositis (JDM). However, only 38% of the patients with JIA, c-SLE, and JDM receiving care at Nationwide Children's Hospital Rheumatology clinic in 2016 had a 25-OHD level ordered in the preceding year. The objective of this project was to increase the percentage of 25-OHD levels ordere…

Antibody Therapies in Autoimmune Inflammatory Myopathies: Promising Treatment Options.

Inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and overlap myositis (OM), in short myositis, are rare diseases. All forms of myositis have progressive muscle weakness in common, with each subtype characterized by different autoantibody profiles, histological findings and extramuscular manifestations. Due to better understanding of the pathogenesis of the muscle inflammation in myositis, new molecular pathways for targeted therapy have been discovered. Current therapies aim at di…

Application value of blood metagenomic next-generation sequencing in patients with connective tissue diseases.

OBJECTIVE: This study aimed to analyze the application value of blood metagenomic next-generation sequencing (mNGS) in patients with connect … conventional diagnostic tests (CDTs). RESULTS: Systemic lupus erythematosus (SLE) and polymyositis/dermatomyositis (DM/PM) had the highest incidence of infections. The positive pathogen detection rates of mNGS were higher than those of CDT. The virus infections are the most common type in CTD patients with single or mixed infection, especially Human gammaherpesv …

Celiac Disease in Juvenile Idiopathic Arthritis and Other Pediatric Rheumatic Disorders.

Celiac Disease (CD) is an immune-mediated and gluten-related disorder whose prevalence is higher in children affected with other autoimmune … affected with other rheumatic diseases (including pediatric systemic lupus erythematosus, juvenile dermatomyositis, and systemic sclerosis).

Cutaneous Immune-Related Adverse Events Secondary to Immune Checkpoint Inhibitors and Their Management.

Immune checkpoint inhibitors (CPIs) are highly effective in the treatment of various cancers. Immunotherapy enhances antitumor activity by r … exacerbation of pre-existing cutaneous autoimmune disease (subacute cutaneous lupus erythematosus, dermatomyositis, eosinophilic fasciitis, leukocytoclastic vasculitis, and scleroderma-like reaction).

T-cell chimerism prior to graft-versus-host disease.

Graft-versus-host disease (GVHD), a serious complication of haematopoietic stem cell transplantation, can occur following solid organ transplantation. However, diagnosing solid organ transplantation-associated GVHD is difficult, and its risk factors are not fully understood. Here, we report a GVHD case in a 59-year-old woman with dermatomyositis-associated interstitial pneumonia, who took immunosuppressants including corticosteroids before receiving right lung transplantation from a 13-year-old brain-dead male donor. She developed systemic erythema with desquamation and pancytopenia by day 20.…

Spontaneous Pneumomediastinum due to Anti-Melanoma Differentiation-Associated Protein 5 Requiring a Bilateral Lung Transplant.

Anti-melanoma differentiation-associated protein 5 (anti-MDA5) is a subset of dermatomyositis associated with respiratory complications, in which rapidly progressive interstitial lung disease (RPILD) is commonly cited, and spontaneous pneumomediastinum (SPM) is a rare complication. In medical literature, aggressive immunosuppressive therapy has been the mainstay of anti-MDA5-associated SPM management. Here, we report the first MDA5 case with SPM which was successfully treated with a double-lung transplant. We present a 48-year-old male who presented with multiple constitutional symptoms such a…

Biomarkers of B cell activation in autoimmune connective tissue diseases: More than markers of disease activity.

B cells play a central role in the pathogenesis of many autoimmune diseases, acting as antigen-presenting cells, producing inflammatory cytokines, and acting as a source of autoantibodies after differentiating into plasma cells. In this review, we aim to summarize and synthesize the literature for the utility of biomarkers of B cell activation (plasma immunoglobulins (Ig), free light chains (FLCs), and beta-2 microglobulin (β2M)) in monitoring inflammatory rheumatic connective tissue diseases, such as Sjogren's syndrome (SS), systemic lupus erythematosus (SLE), dermatomyositis (DM), and system…

Prealbumin Correlates with C-reactive Protein in Rheumatic Musculoskeletal Diseases.

OBJECTIVE: Prealbumin is a blood component tested for nutrition monitoring, which could be affected during inflammation. This study aimed to investigate the relationships between prealbumin and C-reactive protein (CRP) in inflammatory rheumatic musculoskeletal diseases (RMDs), including Takayasu arteritis, antineutrophil cytoplasmic antibody-associated vasculitis, systemic lupus erythematosus, Behcet's disease and polymyositis/dermatomyositis. METHODS: A total of 52 healthy controls and 508 RMD patients were included. We collected 3714 clinical and laboratory records from June 2011 to August …

Neurology of inflammatory bowel disease.

Inflammatory bowel diseases (IBD) are chronic inflammatory conditions affecting the digestive system, comprising two main distinctive entiti … include peripheral neuropathy, either demyelination and axonal, myasthenia gravis and polymyositis/dermatomyositis and localized forms of myositis.