Rheumatic diseases manifest with numerous symptoms and syndromes that reflect musculoskeletal and extra-articular affections and require cross-disciplinary diagnostic, preventive, and therapeutic approaches [1, 2]. The progressive course of the diseases with systemic inflammation, (auto)immune derangements, pain syndromes, and impaired joint mobility results in multiple organ affections and comorbid states [3]. Cardiovascular affections and comorbidities are highly prevalent in patients with rheumatic diseases primarily due to high-grade systemic inflammation and high-dose and lasting corticosteroid therapies [4, 5]. The current thematic collection of reviews in Clinical Rheumatology aims to explore available evidence and pave the way for a new series of studies in the field of cardiovascular rheumatology.

Pathophysiological mechanisms of cardiovascular affections in rheumatic diseases are complex and, at times, irreversible, leading to enhanced atherosclerosis and thrombotic vascular events. Notably, subclinical vascular affections start with the degradation of endothelial glycocalyx due to exposure to reactive blood cells and enzyme (sheddase) activation with a resultant loss of endothelial protection, inflammatory cell migrations, and progressive vascular damage [6]. Vascular damage can be missed at early stages due to the unavailability of clinically validated (bio)markers and standardized visualization techniques.

Although the cardiovascular phenomenon is primarily coined in prototype inflammatory rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, accumulating evidence points to shared features of vascular affections in systemic sclerosis [7, 8], idiopathic inflammatory myopathies [9], spondyloarthropathies [10, 11], and non-inflammatory syndromes such as fibromyalgia [12]. While some of the affections such as pulmonary hypertension and digital ulcers in SSc can be treated with the use of anti-inflammatory and cardiovascular drugs [13, 14], sympathetic nervous system dominance and autonomic dysfunction in spondyloarthropathies and fibromyalgia can be targeted non-pharmacologically [15, 16]. The latter holds promise given the anti-inflammatory and analgesic properties of vagus nerve stimulation and accumulating evidence of the efficiency of its use in combined therapies [17].

Childhood rheumatic diseases represent a distinct group with a variety of cardiovascular manifestations and mortality trends differing from those in adulthood. Although enhanced atherosclerosis with an increased intimal-medial thickness on carotid ultrasound is common in children with inflammatory rheumatic diseases, mortality trends in this age group are primarily determined by pulmonary hypertension in scleroderma, pericardial effusion with cardiac tamponade in lupus and juvenile idiopathic arthritis, and coronary aneurysm with rupture in Kawasaki disease [18]. Early detection of cardiovascular involvement in children is challenging due to the delayed diagnoses and/or insufficiently used sensitive visualization techniques. Timely use of cardiac echography and magnetic resonance imaging may help detect some dreadful cardiovascular complications, but these techniques are still largely unavailable or rarely included in complex diagnostic procedures [19].

Subclinical inflammation-induced atherosclerosis in childhood may transform into carotid plaques and atherothrombotic events in later life [20]. Inappropriate anti-inflammatory therapies and accumulating cardiovascular risk factors may contribute to the risk of thrombotic events with aging. As such, timely detection and modification of risk factors are perhaps the most useful preventive strategy in adults with rheumatic diseases. Counseling patients and motivating them to quit smoking stands out as the most important preventive measure given the results of multiple cohort studies [9]. Monitoring arterial blood pressure, glycemic and lipid profiles, and timely administering anti-diabetic, lipid-lowering, and anti-hypertensive drugs are often recommended to minimize the risk of vascular events in patients with inflammatory rheumatic diseases [10]. Increasing patients’ awareness of modifiable risk factors and exploring specialists’ experiences of tackling enhanced cardiovascular risk may help appropriately revise available practice guidelines [10, 21, 22].

Available prediction tools underestimate or overestimate cardiovascular risk in patients with inflammatory rheumatic diseases [5]. Empirical adjustments of the risk scoring take into account systemic inflammation as a modifier without correcting for diverse autoimmune markers, disease-specific variables, and disease-modifying anti-rheumatic drugs. The patient’s exposure to COVID-19 during the pandemic further complicates the risk prediction at the current stage. COVID-19 increases the risk of atherothrombotic events in rheumatic diseases by triggering multiple pathways of endothelial dysfunction, inflammatory cell activation, and hypercoagulation [23]. Cardiovascular comorbidities in COVID-19 survivors with rheumatic diseases may further complicate the risk prediction. While the outcomes of the pandemic are still overburdening rheumatology services, alerting rheumatologists and allied specialists of the enhanced risk of thromboses and related preventive measures emerges as a strategically important task.

To sum up, cardiovascular manifestations and comorbidities continue to make headlines in mainstream rheumatology journals. The current thematic collection of selected reviews sheds light on some diagnostic, preventive, and therapeutic approaches to rheumatic diseases with cardiovascular manifestations and comorbidities. The key messages of these reviews may guide rheumatologists and allied specialists in their attempts to visualize affections by cardiac magnetic imaging and other sensitive techniques, comprehensively evaluate all organ systems, and target systemic inflammation along with other components of cardiovascular risk. Motivating researchers to generate new hypotheses, survey patients and specialists, explore innovative pharmacological and non-pharmacological approaches, and update available practice guidelines is perhaps the main outcome of this valuable publishing initiative. Enjoy reading, commenting, and disseminating information on this thematic collection!