Cardiovascular disease is the leading cause of morbidity and mortality in patients with rheumatoid arthritis and systemic lupus erythematosus. Traditional cardiovascular risk factors, although present in lupus and rheumatoid arthritis, do not explain such a high burden of early cardiovascular disease in the context of these systemic connective tissue diseases. Over the past few years, our understanding of the pathophysiology of atherosclerosis has changed from it being a lipid-centric to an inflammation-centric process. In this review, we examine the pathogenesis of atherosclerosis in systemic lupus erythematosus and rheumatoid arthritis, the two most common systemic connective tissue diseases, and consider them as emblematic models of the effect of chronic inflammation on the human body. We explore the roles of the inflammasome, cells of the innate and acquired immune system, neutrophils, macrophages, lymphocytes, chemokines and soluble pro-inflammatory cytokines in rheumatoid arthritis and systemic lupus erythematosus, and the roles of certain autoantigens and autoantibodies, such as oxidized low-density lipoprotein and beta2-glycoprotein, which may play a pathogenetic role in atherosclerosis progression.

Background: Myeloproliferative neoplasms (MPNs) are often associated with splanchnic vein thrombosis (SVT). Not all the factors involved in the thrombotic tendency are currently known. Objectives: This study aims to evaluate a possible association between ADAMTS13, von Willebrand factor (VWF), platelet microvesicles (MV), and factor VIII activity (FVIII:C) with thrombotic events in MPN patients. Materials and methods: In total, 36 consecutive MPN patients with SVT were enrolled. The MPNs were diagnosed based on clinical characteristics and one or more gene mutations among JAK-2, CALR, and MPL. As controls, 50 randomly selected patients with MPN without thrombosis, 50 patients with deep vein thrombosis without MPNs, and 50 healthy blood donors were evaluated. Complete blood count, ADAMTS13, VWF, MV, and FVIII:C in plasma were measured in all the subjects. Results: The JAK-2 mutation was found in 94% of the patients with SVT, but none were triple-negative for genetic mutations (JAK2 V617F, CALR, MPL, and exon 12). Compared to the normal subjects, in all the MPN patients (with or without SVT), the levels of ADAMTS13 were found to be significantly lower (p < 0.001) and the MV concentrations were significantly higher (p < 0.001). Among the MPN patients, the VWF and FVIII:C levels were significantly higher in the patients with SVT than those without thrombosis (p = 0.007 and p = 0.04, respectively). Splenomegaly was present in 78% of MPN patients with SVT and in 30% of those without SVT (p < 0.001). The ADAMTS13/VWF ratio was reduced in all the patients, but not in the healthy blood donors (p < 0.001). Conclusions: The significant increase in circulating MV, VWF, and FVIII:C in the MPN patients and in the patients with thrombosis supports the role of endothelium damage in promoting thrombotic events. In particular, a significant increase in VWF and FVIII:C levels was found in the MPN patients with SVT.

Sodium–glucose cotransporter-2 inhibitors (SGLT2i) are a specific class of drugs originally developed for treating type 2 diabetes mellitus. Subsequently, studies demonstrated that their action was not limited to glycemic control but could also have positive effects on other specific outcomes, particularly at the cardiovascular level. Indeed, due to their diuretic effect, SGLT2i improve the clinical control of chronic heart failure and reduce the risk of rehospitalization. In addition, other studies reported a protective effect on major cardiovascular events and mortality. More recently, it has been suggested that the prescription of SGLT2i after an acute myocardial infarction may have positive effects due to their possible effect on inflammation, arrhythmias, and ventricular remodeling. Here, we reviewed studies focused on SGLT2i after an acute myocardial infarction in patients treated with percutaneous coronary intervention.

Inborn errors of immunity (IEI) are multifaced diseases which can present with a variety of phenotypes, ranging from infections to autoimmunity, lymphoproliferation, and neoplasms. In recent decades, research has investigated the relationship between autoimmunity and IEI. Autoimmunity is more prevalent in primary humoral immunodeficiencies than in most other IEI and it can even be their first manifestation. Among these, the two most common primary immunodeficiencies are selective IgA deficiency and common variable immunodeficiency. More than half of the patients with these conditions develop non-infectious complications due to immune dysregulation: autoimmune, autoinflammatory, allergic disorders, and malignancies. Around 30% of these patients present with autoimmune phenomena, such as cytopenia, gastrointestinal and respiratory complications, and endocrine and dermatologic features. Complex alterations of the central and peripheral mechanisms of tolerance are involved, affecting mainly B lymphocytes but also T cells and cytokines. Not only the immunophenotype but also advances in genetics allow us to diagnose monogenic variants of these diseases and to investigate the pathogenetic basis of the immune dysregulation. The diagnosis and therapy of the primary humoral immunodeficiencies has been mostly focused on the infectious complications, while patients with predominant features of immune dysregulation and autoimmunity still present a challenge for the clinician and an opportunity for pathogenetic and therapeutic research.

Introduction: Kounis syndrome is an acute coronary syndrome precipitated by allergic or hypersensitivity reactions and mediated by the release of inflammatory substances that affect the coronary circulation. In spite of increasing awareness of this syndrome, it remains underdiagnosed, particularly in young patients without conventional cardiovascular risk factors. Case description: We report the case of a patient with recurrent Kounis syndrome presenting with two distinct acute coronary syndromes within one month, initially classified as myocardial infarction with nonobstructive coronary arteries and subsequently recognised after angiographic documentation of transient coronary vasospasm. A 28-year-old man with a history of non-steroidal anti-inflammatory drug (NSAID) hypersensitivity presented with acute chest pain shortly after NSAID intake. Electrocardiography showed ischemic changes, and cardiac biomarkers showed a modest elevation. Coronary angiography revealed severe, reversible multivessel coronary artery spasm in the absence of structural coronary disease, consistent with type I Kounis syndrome. During the procedure, the patient developed ventricular fibrillation, successfully treated with electrical defibrillation and intracoronary nitrates. Left ventricular function fully recovered, and the patient was discharged with strict avoidance of NSAIDs. Subsequent allergological evaluation confirmed NSAID hypersensitivity. Conclusion: This case highlights that type I Kounis syndrome may present as a severe acute coronary syndrome complicated by malignant ventricular arrhythmias. A careful allergological and pharmacological history is essential for accurate diagnosis, guide management, and prevent recurrent potentially fatal events.