In the ever evolving landscape of systemic immune mediated diseases, an increased awareness regarding the associated cardiovascular system impairment has been noted in recent years. Even though primary Sjögren’s Syndrome (pSS) is one of the most frequent autoimmune diseases affecting middle-aged individuals, the cardiovascular profile of this specific population is far less studied, at least compared to other autoimmune diseases. Traditional cardiovascular risk factors and disease specific risk factors are inextricably intertwined in this particular case. Therefore, the cardiovascular risk profile in pSS is a multifaceted issue, sometimes difficult to assess. Furthermore, in the era of multimodality imaging, the diagnosis of subclinical myocardial and vascular damage is possible, with recent data pointing that the prevalence of such involvement is higher in pSS than in the general population. Nevertheless, when approaching patients with pSS in terms of cardiovascular diseases, clinicians are often faced with the difficult task of translating data from the literature into their everyday practice. The present review aims to synthesize the existing evidence on pSS associated cardiovascular changes in a clinically relevant manner.

Extraglandular manifestations (EGMs) in primary Sjogren’s syndrome (pSS) represent the clinical expression of the systemic involvement in this disease. EGMs are characterized by a wide heterogeneity; virtually any organ or system can be affected, with various degrees of dysfunction. The existing gaps of knowledge in this complex domain of extraglandular extension in pSS need to be overcome in order to increase the diagnostic accuracy of EGMs in pSS. The timely identification of EGMs, as early as from subclinical stages, can be facilitated using highly specific biomarkers, thus preventing decompensated disease and severe complications. To date, there is no general consensus on the diagnostic criteria for the wide range of extraglandular involvement in pSS, which associates important underdiagnosing of EGMs, subsequent undertreatment and progression to severe organ dysfunction in these patients. This review article presents the most recent basic and clinical science research conducted to investigate pathogenic mechanisms leading to EGMs in pSS patients. In addition, it presents the current diagnostic and treatment recommendations and the trends for future therapeutic strategies based on personalized treatment, as well as the latest research in the field of diagnostic and prognostic biomarkers for extraglandular involvement in pSS.

Background and Objectives: Romania has one of the highest prevalence of hepatitis B virus (HBV) infection in human immunodeficiency virus (HIV) patients, mostly in those parenterally infected during childhood; nevertheless, there are scarce data on the virological profile of co-infection. The objective of this study was to assess the prevalence of HBV genotypes and antiviral resistance-associated mutations (RAMs) in these co-infected patients, in order to monitor the viral factors associated with the evolution of liver disease. Materials and Methods: HBV genotypes and RAMs were detected using nested PCR and line probe assays (INNO-LiPA HBV genotyping assay, and INNO-LiPA HBV DR v2, Innogenetics). Results: Out of 117 co-infected patients, 73.5% had detectable HBV-DNA, but only 38.5% presented an HBV viral load >1000 IU/mL. HBV genotype A was present in 66.7% of the cases and was dominant in patients parenterally infected during early childhood, who experienced multiple treatment regimens, with a mean therapy length of 15.25 years, and present numerous mutations associated with lamivudine (LAM) resistance, but very rarely active liver disease. HBV genotype D was detected in 33.3% of the cases, mostly in recently diagnosed injecting drug users who are treatment naïve, but, nevertheless, present RAMs in 63.5% of the cases, suggesting transmitted drug resistance, and display more frequently advanced liver fibrosis (36.1% vs. 12.3%; p = 0.033). The most frequently encountered RAMs are M204V/I: 48.8%, L180M: 33.3%, L80V: 28.8%, and V173L: 42.2%. There are no significant differences in the distribution of RAMs in patients infected with different HBV genotypes, except for the L80V and N236T mutations, which were more frequently found in HBV genotype A infections (p = 0.032 and p = 0.004, respectively). Conclusions: HBV genotypes A and D are the only genotypes present in HIV–HBV co-infected patients from Romania, with different distributions according to the infection route, and are frequently associated with multiple RAMs, conferring extensive resistance to LAM.

Objective:The case highlights the importance of specific non-traditional cardiovascular risk factors in a patient with premature severe cardiovascular disease, despite an apparently adequate control of traditional cardiovascular risk factors.Design and method:Clinical Case.Results:We present the case of a 38 year-old female with Turner syndrome, with cardiovascular risk factors (mild dislypidemia, type 2 diabetes, grade 1 ESC/ ESH hypertension) who presents to the emergency department for newly-onset constrictive chest pain. Her chronic treatment consisted of a low-dose ACEi and metformin. Clinical exam was unremarkable, except for specific clinical phenotype, with normal BP values in both arms and without any signs of congestion. The electrocardiogram showed sinus rhythm of 65 beats/min, narrow QRS, negative T waves in inferior leads, as well a Q wave in lead III. Hs- troponin (1046pg/ml, ULN: 10pg/ml) and NT-proBNP levels (820,9pg/ml) were elevated. Echocardiography revealed a normal ejection fraction, with localized hypokinesia of the inferior wall and no significant valvular disease. The aortic valve was tricuspid and no signs of aortic coarctaction were observed from the suprasternal view. Coronarography was performed, showing significant CAD, with multiple lesions on ADA, LCXA and RCA. The two suboclusive lesions from the RCA were addressed using two drug eluting stents. The evolution was favourable under specific treatment. The baseline levels of LDLc (117 mg/dl) and glycosylated haemoglobin (5,7%) were not as high as we initially expected, given the severe CAD. Furthermore, the ambulatory 24 h BP monitoring after discharge confirmed that the previous treatment with low-dose ACEi was efficient in maintaining the target BP values.Conclusions:Turner syndrome is associated with a variety of cardiovascular manifestations, such as aortic coarctation, which leads to secondary hypertension, as well as an increased risk of cardiovascular events. In our patient, the hypertension was essential, without any arguments for secondary hypertension, as we would have expected. Furthermore, despite the fact the patient exhibited several traditional cardiovascular risk factors, they were controlled within the recommended targets (with the exception of LDLc, slightly higher than 100 mg/dl). The disease duration appears to be critical, as the patient was diagnosed with hypertension more than 15 years ago. We concluded that the advanced premature CAD is most likely the result of the hormonal imbalance and lack of hormone replacement therapy, for which the patient was no longer eligible. Drastic control of traditional cardiovascular risk factors in this case will probably prove to be insufficient.