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.

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.