Cardiovascular complications in patients with idiopathic inflammatory myopathies: does heart matter in idiopathic inflammatory myopathies?

This review presents a detailed study of original researches and previously published reviews concerning cardiovascular involvement in idiopathic inflammatory myopathies (IIM). We aimed to summarize the current knowledge on the cardiac involvement in IIM, evaluate its impact on mortality and indicate areas still awaiting to be investigated. We searched MEDLINE database (until January 2019) and the reference lists of articles. Selection criteria included only published data, available in English, both original researches and reviews. Articles related to cardiovascular involvement in IIM were selected and analysed. The references were also screened, and relevant articles were included. Cardiovascular involvement is frequent in IIM but typically remains subclinical. Among far less prevalent symptomatic forms, congestive heart failure is the most common. Myocardium and conduction system seems to be predominantly affected. High rate of left ventricular diastolic dysfunction was observed. Non-specific changes of ST-T segment were the most common abnormalities in electrocardiography. Patients with IIM were more frequently affected by atrial fibrillation as compared with other autoimmune diseases. Increased risk of myocardial infarction was observed; furthermore, patients often develop comorbidities that enhance cardiovascular risk. Since cardiovascular disorders remain one of the major causes of death and subclinical involvement is frequent, active screening is justified. Growing availability of the novel imaging techniques may facilitate diagnosis. Correlation between myocardial involvement and the type of autoantibodies and impact of different therapeutic options on the progression of cardiovascular lesions require further studies.

Disability in idiopathic inflammatory myopathies: questionnaire-based study.

Idiopathic inflammatory myopathies (IIM) are progressive, debilitating diseases that can lead to severe impairment. The aim of the study was to evaluate the level of disability and compare it between different subtypes of IIM as well as to estimate clinical symptoms associated with greater risk of disability and distinguish the most troublesome activities in this group of patients. A online form concerning clinical symptoms, comorbidities and limitations in daily living was created and distributed to online support groups for patients with IIM. Health Assessment Questionnaire was used to estimate disability and physical limitations while visual analogue scales enabled to assess the intensity of clinical symptoms. 361 out of 377 responders were included for further evaluation. High prevalence of disability was observed in each subtype yet predominantly in patients with inclusion body myositis (IBM) as 51.43% of them fulfilled the criteria of severe to very severe disability. Level of disability correlated with muscle weakness, tolerance of physical activity and level of fatigue. 45.62% of responders in general required walking devices and 43.50% of participants declared using facilitating devices for maintaining hygiene. Patients with IIM encounter multitude physical limitations that can be partially compensated by usage of facilitating devices or aid of the caregivers. IBM seems to be the most disabling subtype.

From blood coagulation to innate and adaptive immunity: the role of platelets in the physiology and pathology of autoimmune disorders.

Thrombosis and cardiovascular complications are common manifestations of a variety of pathological conditions, including infections and chronic inflammatory diseases. Hence, there is great interest in determining the hitherto unforeseen immune role of the main blood coagulation executor-the platelet. Platelets store and release a plethora of immunoactive molecules, generate microparticles, and interact with cells classically belonging to the immune system. The observed effects of platelet involvement in immune processes, especially in autoimmune diseases, are conflicting-from inciting inflammation to mediating its resolution. An in-depth understanding of the role of platelets in inflammation and immunity could open new therapeutic pathways for patients with autoimmune disorders. This review aims to summarize the current knowledge on the role of platelets in the patomechanisms of autoimmune disorders and suggests directions for future research.

Highlighting the versatility of the citrullination process.

Protein citrullination is one of the processes of post-translational modification of proteins and peptides, playing a crucial role in the homeostasis of important processes, however also leading to pathological processes in autoimmune and neoplastic disorders. Citrullination regulates epidermal function, reproduction being also involved in NET formation. Nowadays, the knowledge about citrullination spreads and opens up a new field for investigation of compounds that potentially be used as a treatment. Inhibitors of PADI are currently being tested in various diseases. As these enzymes play an essential role in the disease progression, one should also look for the basics of regulation of their expression in the cell as well as focus on environmental factors like air pollution or smoking, which can augment the process of citrullination. Despite its pathological properties, citrullination can be a prognostic marker of diseases and a good target for therapies. In this revision, we used PubMed, PubMed Central (PMC), and Google Scholar to collect data which presents information from severalarticles, reviews, case reports, case studies, cohort studies, and clinical trials. Studies conducted in vitro, in vivo on mouse models, postmortem tissue models of animal and human origin were included and non-english articles were excluded. We mainly used keywords like "PAD", Peptidylarginine deiminases", "citrullination" or "deimination" in the search. This revision contains description using both original and review papers can be useful for doctors, scientists, and patients.

Antisynthetase syndrome - much more than just a myopathy.

The aim of the study was to summarize current knowledge on antisynthetase syndrome (ASS), including its epidemiology, pathogenesis, proposed so far diagnostic criteria, heterogeneity of clinical manifestations, prognostic factors and therapeutic possibilities. PubMed database was screened for "antisynthetase syndrome" OR "antisynthetase antibodies" between February and April 2020. Aminoacyl-tRNA synthetases participate in the immune system activation as antigens, but also serve chemoattractive and cytokine-resembling roles, initiating innate and adaptive pathways. Exposure to various inhaled antigens may induce the autoimmune cascade leading to ASS. NK cells with its impaired INF-y production as well as formation of NETs by neutrophils contribute to pathogenesis. The prevalence of symptoms vary significantly depending on the study with muscular, articular and pulmonary involvement being the most frequently observed. Although classified as subtype of idiopathic inflammatory myopathies, myositis may not necessarily be the prominent manifestation. Since clinical presentation is heterogeneous and symptoms can emerge gradually, ASS could be considered as a heterogeneous spectrum rather than a homogenous disease entity. The currently available classification criteria do not fully correspond with the clinical patterns of the disease. Therapy is based on glucocorticosteroids and other immunosuppressive agents. Randomized controlled trials, dedicated for patients with ASS, are needed to form treatment algorithms.

Refractory Angina-Unsolved Problem.

The article discusses pharmacologic and interventional therapeutic options for patients with refractory angina. Refractory angina refers to long-lasting symptoms (≥3 months) due to established reversible ischemia in the presence of obstructive coronary artery disease, which cannot be controlled by escalating medical therapy with second-line and third-line pharmacologic agents, bypass grafting, or stenting. Due to an aging population, increased number of comorbidities, and advances in coronary artery disease treatment, incidence of refractory angina is growing. Although the number of therapeutic options is increasing, there is a lack of randomized clinical trials that could help create recommendations for this group of patients.