Nov 14th, 2022 - Background/Purpose: Antibodies to Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) have been reported in pooled donor plasma and intravenous immunoglobulin products (IVIG) since May 2020 (C Romero 2021 PMID 33606999). It is not known whether regular administration of IVIG increases circulating anti-SARS-CoV-2 antibodies (COVID ab). The current work evaluates COVID ab titers in patie...

Nov 14th, 2022 - Background/Purpose: Nailfold videocapillaroscopy (NVC) is a non-invasive tool used to evaluate the microcirculation of patients with connective tissue diseases (CTDs). A specific combination of NVC abnormalities, known as the “scleroderma pattern,” has been well-described in patients with systemic sclerosis and demonstrated to have prognostic significance. The significance of these changes in p...

Nov 14th, 2022 - Background/Purpose: Idiopathic inflammatory myopathies (IIM) comprise a heterogenous group of acquired autoimmune diseases characterised by inflammation of muscle and affection of other organs, including lung and skin. Some cases of IIM are non-responsive to conventional treatment with glucocorticoids and DMARDs requiring treatment escalation. Only limited data on efficacy and safety of immune-...

Nov 14th, 2022 - Background/Purpose: Anti-transcriptional intermediary factor 1γ (anti-TIF 1γ) antibody is robustly linked with malignancy-associated dermatomyositis (DM) in adults, but its specificity varies widely between series. TIF 1γ can act either as a tumor promoter or suppressor and may act as an autoantigen. To investigate the association between anti-TIF 1γ antibody positivity and the development of n...

Nov 14th, 2022 - Background/Purpose: Patients with dermatomyositis (DM) and antibodies (ab) to melanoma differentiation associated gene 5 (MDA5) share similar clinical characteristics to patients infected with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) including high risk of fatal, rapidly progressive interstitial lung disease (RP-ILD). Nearly 50% of patients with COVID 19 have anti-MDA5 a...

Nov 14th, 2022 - Background/Purpose: In some patients, interstitial lung disease (ILD) may be the dominant or even sole overt manifestation of an otherwise unrecognised autoimmune connective tissue disease (CTD). Accurate diagnosis can be challenging given considerable overlap of the clinical, radiological and histological disease features. Distinguishing CTD related ILD from idiopathic ILD, enables appropriate...

Nov 14th, 2022 - Background/Purpose: Idiopathic inflammatory myositis (IIM) includes a spectrum of a rare autoimmune disease characterized by proximal muscle weakness, variable skin manifestation and extra muscular manifestations. Rituximab has been shown to be effective in refractory IIM. The aim was to describe myositis specific autoantibodies (MSA) associated clinical and laboratory characteristics including...

Nov 14th, 2022 - Background/Purpose: Muscle inflammation is a major clinical manifestation in patients with polymyositis/dermatomyositis (PM/DM). Phenotypes of PM/DM patients, especially those with interstitial lung diseases, are known to be classified through different subsets of autoantibodies, however, there are few studies evaluating muscle inflammation by serum biomarkers. The aim of this study is to ident...

Nov 14th, 2022 - Background/Purpose: Patients with comorbidities and active rheumatic disease have increased morbidity and hospitalization following SARS-CoV-2 infection. While vaccination has decreased this, many unknown factors still influence COVID-19 vaccine hesitancy. The data on predictors of vaccine hesitancy is regional and scarce. We aimed to analyze the factors influencing vaccine hesitancy in 2022 an...

Nov 14th, 2022 - Background/Purpose: Muscle atrophy is mediated by the ubiquitination of myofilaments by two ubiquitin ligases called Tripartite Motif Containing (TRIM) 63 and Atrogin-1, which are induced by inflammation and interferons (IFN). Muscle atrophy is the main cause of disability in patients with idiopathic inflammatory myopathies (IIM), but notwithstanding its relevance, the expression of TRIM63 and ...

Nov 14th, 2022 - Background/Purpose: B Cell Activating Factor (BAFF) is a cytokine that drives B Cell proliferation and maturation. B Cells remain an important factor in JDM immune pathophysiology through the production of pathogenic autoantibodies. BAFF has been previously evaluated in the Japanese MDA5-JDM population and found to associate with the presence of rapidly progressive ILD (interstitial lung diseas...

Nov 14th, 2022 - Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of disorders in which autoimmune responses produce a chronic state of inflammation resulting in degeneration of skeletal muscle structure and function. Our previous work generated a robust model of inflammatory myositis by combining a mouse with impaired sarcolemmal membrane repair synaptotagmin VII (SytVII)-/- with a mous...

Nov 14th, 2022 - Background/Purpose: Nipocalimab, a fully human, effectorless IgG1 anti-neonatal Fc receptor (FcRn) monoclonal antibody, binds to FcRn with high affinity which prevents IgG recycling, leading to reduced serum levels of total IgGs, including pathogenic IgG autoantibodies. Rapid, sustained lowering of IgG was observed in the phase 2 VIVACITY study in generalized myasthenia gravis (gMG) and in phas...

Nov 14th, 2022 - Background/Purpose: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily affects the skin and lung; few effective treatment options are available. Lenabasum is a cannabinoid type 2 receptor agonist that activates the resolution phase of inflammation. Results from DETERMINE, a Phase 3 trial of lenabasum, demonstrated improvement in CDASI activity (CDASI-A) scores with t...

Nov 14th, 2022 - Background/Purpose: Juvenile Dermatomyositis(JDM) is heterogenous disease with many presentations. The underlying immune pathophysiology of JDM remains complex with varying phenotypes. B Cells remain a critical element in the pathophysiology both through the production of pathogenic antibodies but also as a target for the treatment of JDM. In order to better understand the role of B Cells in JD...

Nov 14th, 2022 - Background/Purpose: Dermatomyositis (DM) is a systemic autoimmune disease that affects the skin and muscle. Lenabasum, a cannabinoid type 2 receptor (CB2R) agonist, has been developed as a potential treatment to reduce inflammation in DM. Additional research has also shown that lenabasum also exerts its anti-inflammatory effects through the binding of peroxisome proliferator-activated receptor ...

Nov 14th, 2022 - Background/Purpose: Interstitial Lung Disease (ILD) accompanied with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (DM) is usually rapidly progressive and life-threatening with poor prognosis. Although serum ferritin has been reported as a prognostic marker for ILD among patients with anti-MDA5 antibody positive DM, serum ferritin levels does not neces...

Nov 14th, 2022 - Background/Purpose: Polymyositis (PM)/ Dermatomyositis (DM) is a chronic immune-mediated myositis characterized by relapses that can lead to frequent hospitalizations. There is a scarcity of national population-based studies on PM)/DM readmissions in the United States (US). In this study, we aim to describe the rates, reasons for readmissions, and characteristics of readmissions for adults hosp...

Nov 14th, 2022 - Background/Purpose: A lack of education in dermatopathology of non-white skin is of growing concern in the field of medical education. Diseases such as Dermatomyositis (DM) with pathognomonic dermatological features rely on swift recognition for diagnosis and treatment. There are no studies comparing detection and treatment initiation in patients with DM of different races and ethnicities. Our ...

Nov 14th, 2022 - Background/Purpose: Patients with CTD-ILD are often treated with a combination of corticosteroids and steroid sparing agents to limit the progression of the inflammatory response in the lung and prevent remodeling that can lead to fibrosis. While tapering regimens for steroids are part of the standard of care in CTD-ILD, there is no significant experience on outcomes in patients who taper stero...