Retroperitoneal fibrosis: a case of a patient (63y/o) treated with low-dose methotrexate (MTX) and 6-methylprednisolone (6-MP)

Retroperitoneal fibrosis (RPF), is a rare fibroinflammatory disease. The pathogenesis of RPF is still unclear and numerous theories have been reported such as environmental factors, immunologic process, genetic component, local inflammation and advanced atherosclerosis. RPF is characterized by the presence of a particular retroperitoneal fibrotic tissue which is white, woody and involving retroperitoneal structures such as the great vessels, ureters and psoas muscle. The main complication of RPF is the obstruction of local structures such as the ureters due to the fibrosis and the treatment of this aspect represents the main challenge for this pathology. RPF medical treatment consists of corticosteroids or/and immunosuppressive therapy. We report a case of a patient (63y/o) affected by idiopathic RPF treated with low-dose methotrexate (MTX) and 6-methylprednisolone (6-MP) for two years, describing and confirming the effectiveness and safety of a long-term low-dose MTX and 6-MP treatment

Elevated serum levels of macrophage migration inhibitory factor and stem cell growth factor β in patients with idiopathic and systemic sclerosis associated pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) can be idiopathic or secondary to autoimmune diseases, and it represents one of the most threatening complications of systemic sclerosis (SSc). Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine with proinflammatory functions that appears to be involved in the pathogenesis of hypoxia-induced PH. In SSc patients, high serum levels of MIF have been associated with the development of ulcers and PAH. Stem cell growth factor β (SCGF β) is a human growth factor that, together with MIF, is involved in the pathogenesis of chronic spinal cord injury. The aim of our study was to measure serum levels of MIF in patients with idiopathic and SSc-associated PAH. We enrolled 13 patients with idiopathic PAH and 15 with SSc-associated PAH. We also selected 14 SSc patients without PAH and 12 normal healthy controls, matched for sex and age. PAH was confirmed by right hearth catheterism (mPAP>25 mmHg). MIF and SCGF β levels were measured by ELISA. We found significantly higher circulating levels of MIF and of SCGF β in patients with idiopathic PAH (P=0.03 and P=0.004) and with PAH secondary to SSc (P=0.018 and P=0.023) compared to SSc patients without PAH. Higher levels of MIF were found in those patients with an higher New York Heart Association (NYHA) class (P=0.03). We can hypothesize that MIF and SCGF β are able to play a role in PAH, both idiopathic or secondary, and in the future they may be evaluated as useful biomarkers and prognostic factors for this serious vascular disease

Echocardiography combined with cardiopulmonary exercise testing for the prediction of outcome in idiopathic pulmonary arterial hypertension

BACKGROUND: Right ventricular (RV) function is a major determinant of exercise intolerance and outcome in idiopathic pulmonary arterial hypertension (IPAH). The aim of the study was to evaluate the incremental prognostic value of echocardiography of the RV and cardiopulmonary exercise testing (CPET) on long-term prognosis in these patients. METHODS: One hundred-thirty treatment-naïve IPAH patients were enrolled and prospectively followed. Clinical worsening (CW) was defined by a reduction in 6-minute walk distance plus an increase in functional class, or non elective hospitalization for PAH, or death. Baseline evaluation included clinical, hemodynamic, echocardiographic and CPET variables. Cox regression modeling with c-statistic and bootstrapping validation methods were done. RESULTS: During a mean period of 528 ± 304 days, 54 patients experienced CW (53%). Among demographic, clinical and hemodynamic variables at catheterization, functional class and cardiac index were independent predictors of CW (Model-1). With addition of echocardiographic and CPET variables (Model-2), peak O2 pulse (peak VO2/heart rate) and RV fractional area change (RVFAC) independently improved the power of the prognostic model (AUC: 0.81 vs 0.66, respectively; p=0.005). Patients with low RVFAC and low O2 pulse (low RVFAC + low O2 pulse) and high RVFAC+low O2 pulse showed 99.8 and 29.4 increase in the hazard ratio, respectively (relative risk -RR- of 41.1 and 25.3, respectively), compared with high RVFAC+high O2 pulse (p=0.0001). CONCLUSIONS: Echocardiography combined with CPET provides relevant clinical and prognostic information. A combination of low RVFAC and low O2 pulse identifies patients at a particularly high risk of clinical deterioration

The Impact of School and After-School Friendship Networks on Adolescent Vaccination Behavior

Psychological and social characteristics of individuals are important determinants of their health choices and behaviors. Social networks represent "pipes" through which information and opinions circulate and spread out in the social circle surrounding individuals, influencing their propensity toward important health care interventions. This paper aims to explore the relationship between students' vaccination health choices and their social networks. We administered a questionnaire to students to collect data on individual students' demographics, knowledge, and attitudes about vaccinations, as well as their social networks. Forty-nine pupils belonging to 4 classrooms in an Italian secondary school were enrolled in the study. We applied a logistic regression quadratic assignment procedure (LR-QAP) by regressing students' positive responsive behavior similarity as a dependent variable. LRQAP findings indicate that students' vaccination behavior similarity is significantly associated with after-school social ties and related social mechanisms, suggesting that pupils are more likely to share information and knowledge about health behaviors through social relationships maintained after school hours rather than through those established during the school day. Moreover, we found that vaccination behaviors are more similar for those students having the same ethnicity as well as for those belonging to the same class. Our findings may help policymakers in implementing effective vaccination strategies

Therapeutic Strategies in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a serious and life-threatening condition for which the prognosis remains poor. Treatment options include endothelial receptor antagonists, phosphodiesterase (PDE5) inhibitors and prostanoids. Despite all demonstrating good short-term efficacy, none of the currently available drug therapies are curative. Treatment with prostanoids is complex and requires careful monitoring and management through a specialist centre. Furthermore, clinical efficacy is dependent on adequate up-titration of the drug. Treatment should be individualised and modified according to clinical response, with the addition of other therapies if required. The importance of monitoring and modifying therapeutic regimes is discussed. There appears to be reluctance among patients and physicians to employ prostanoid therapy, though an aggressive first-line therapy may be appropriate in advanced cases

The sustainability of influenza vaccination program: considerations and perspectives from Italy.

Influenza represents an annual recurring threat to society, both from clinical and economic point of view. The impact of influenza is often underestimated, especially among frail elderly people who are at increased risk for serious complications, including hospitalization and death. In Italy there are around 10 million individuals 65 years and older who are at risk of contracting influenza and it is possible to estimate that the absence of a vaccination strategy would lead to more than 2 million cases and about 30,000 deaths. However, the uptake of routinely recommended adult immunizations remains suboptimal despite the availability of safe and effective vaccines. A monitoring programme from the National Institute of Health in Italy has shown that influenza vaccination coverage in the elderly dropped to 49% in the 2014-2015 season, which is far less than maximum values (68%) detected in the 2005-2006 season.  The current situation in Italy imposes a need for a new sustainability, which would be able to meet the challenges related to the evolution of epidemiological situation, demographic transition and great social transformations. Our review sums up the key elements of influenza vaccine sustainability and gives suggestions for improving the organizational structure of the present initiatives.

Italian immunization calendar implementation: Time to optimize number of vaccination appointments?

In the Italian vaccination schedule, at least six vaccination appointments are scheduled in the first year of life. This implies more discomfort for both the patient and the parents. This was particularly evident during the COVID-19 pandemic, during which several appointments were missed. A UK experience with three injectable vaccines and an oral one co-administered at the same appointment (4-in-1) at 2 and 4 months of age showed interesting results. The vaccination coverage was high, consistent with previous practice, and no relevant increase in adverse events was reported. Translating the UK experience into the Italian context would not be immediate, due to several organizational and social issues. Nevertheless, this option warrants some further considerations, which are discussed in this manuscript