Common Variable Immunodeficiency in Elderly Patients: A Long-Term Clinical Experience

none7siAbstract: Background: Common variable immunodeficiency (CVID) is a complex, predominantly antibody deficiency usually diagnosed between 20–40 years. Few data about elderly patients are reported in the literature. Our aim was to evaluate the clinical phenotypes of elderly patients with CVID. Method: A retrospective analysis of adult patients with CVID was performed in our Referral Centre, focusing on the main differences between “older” patients (≥65 years at the diagnosis) and “younger” patients (<65 years). Results: The data from 65 younger and 13 older patients followed up for a median period of 8.5 years were available. At diagnosis, recurrent infections represented the only clinical manifestation in 61% and 69% of younger and older patients, respectively. The incidence of autoimmune diseases was higher in elderly patients compared with younger ones (30 vs. 18%, respectively). During the follow-up, the incidence of autoimmune disorders and enteropathy increased in the younger patients whereas neoplasia became the most prevalent complication in the elderly (38%). All patients received a replacement therapy with immunoglobulin, with good compliance. Conclusion: CVID occurrence in elderly patients is rarely described; therefore, the clinical characteristics are not completely known. In our series, neoplasia became the most prevalent complication in the elderly during the follow-up. In elderly patients, 20% SCIg was as safe as in the younger ones, with good compliance. A genetic analysis is important to confirm the diagnosis, identify specific presentations in the different ages, clarify the prognosis and guide the treatment. Future clinical research in this field may potentially help to guide their care.openDanieli, Maria Giovanna; Mezzanotte, Cristina; Verga, Jacopo Umberto; Menghini, Denise; Pedini, Veronica; Bilò, Maria Beatrice; Moroncini, GianlucaDanieli, Maria Giovanna; Mezzanotte, Cristina; Verga, Jacopo Umberto; Menghini, Denise; Pedini, Veronica; Bilò, Maria Beatrice; Moroncini, Gianluc

Acute Delta Hepatitis in Italy spanning three decades (1991–2019): Evidence for the effectiveness of the hepatitis B vaccination campaign

Updated incidence data of acute Delta virus hepatitis (HDV) are lacking worldwide. Our aim was to evaluate incidence of and risk factors for acute HDV in Italy after the introduction of the compulsory vaccination against hepatitis B virus (HBV) in 1991. Data were obtained from the National Surveillance System of acute viral hepatitis (SEIEVA). Independent predictors of HDV were assessed by logistic-regression analysis. The incidence of acute HDV per 1-million population declined from 3.2 cases in 1987 to 0.04 in 2019, parallel to that of acute HBV per 100,000 from 10.0 to 0.39 cases during the same period. The median age of cases increased from 27 years in the decade 1991-1999 to 44 years in the decade 2010-2019 (p &lt; .001). Over the same period, the male/female ratio decreased from 3.8 to 2.1, the proportion of coinfections increased from 55% to 75% (p = .003) and that of HBsAg positive acute hepatitis tested for by IgM anti-HDV linearly decreased from 50.1% to 34.1% (p &lt; .001). People born abroad accounted for 24.6% of cases in 2004-2010 and 32.1% in 2011-2019. In the period 2010-2019, risky sexual behaviour (O.R. 4.2; 95%CI: 1.4-12.8) was the sole independent predictor of acute HDV; conversely intravenous drug use was no longer associated (O.R. 1.25; 95%CI: 0.15-10.22) with this. In conclusion, HBV vaccination was an effective measure to control acute HDV. Intravenous drug use is no longer an efficient mode of HDV spread. Testing for IgM-anti HDV is a grey area requiring alert. Acute HDV in foreigners should be monitored in the years to come

Intravenous immunoglobulin as an important adjunct in the prevention and therapy of coronavirus 2019 disease

The coronavirus disease-19 (COVID-19) pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) challenged globally with its morbidity and mortality. A small percentage of affected patients (20%) progress into the second stage of the disease clinically presenting with severe or fatal involvement of lung, heart, and vascular system, all contributing to multiple-organ failure. The so-called “cytokines storm" is considered the pathogenic basis of severe disease and it is a target for treatment with corticosteroids, immunotherapies, and intravenous immunoglobulin (IVIg). We provide an overview of the role of IVIg in the therapy of adult patients with COVID-19 disease. After discussing the possible underlying mechanisms of IVIg immunomodulation in COVID-19 disease, we review the studies in which IVIg was employed. Considering the latest evidence that show a link between new coronavirus and autoimmunity, we also discuss the use of IVIg in COVID-19 and anti-SARS-CoV-2 vaccination related autoimmune diseases and the post-COVID-19 syndrome. The benefit of high-dose IVIg is evident in almost all studies with a rapid response, a reduction in mortality and improved pulmonary function in critically ill COVID-19 patients. It seems that an early administration of IVIg is crucial for a successful outcome. Studies limitations are represented by the small number of patients, the lack of control groups in some and the heterogeneity of included patients. IVIg treatment can reduce the stay in ICU and the demand for mechanical ventilation, thus contributing to attenuate the burden of the disease

A Case of CVID-Associated Inflammatory Bowel Disease with CTLA-4 Mutation Treated with Abatacept

CTLA-4 (cytotoxic T-lymphocyte-associated protein 4) deficiency is a widely known cause of immunodeficiency and autoimmunity, determining an immune dysregulation syndrome. We here present the case of a Caucasian woman with common variable immunodeficiency (CVID) and severe enteropathy secondary to CTLA4 deficiency, successfully treated with abatacept. Case Report: A 54-year-old woman affected by CVID, treated with 20%subcutaneous immunoglobulin, came to our attention for chronic diarrhoea, severe malabsorption and significant weight loss, requiring parenteral nutrition. Her past clinical history comprised psoriatic arthritis, idiopathic thrombocytopenic purpura and multifactorial anemia. She started treatment with glucocorticoids and adalimumab, with partial benefit. However, after six months anti-TNF-alpha agent was withdrawn for recurrent serious infectious episodes. Due to the complex phenotype, we performed a genetic analysis, which revealed the presence of a CTLA-4 heterozygous mutation, with consequent CTLA-4 deficiency. We decided therefore to start abatacept, a CTLA-4 fusion protein, at the dose of 125 mg/week subcutaneously in association with budesonide, with improvement. At one year of follow-up diarrhea is still in remission and the patient has gained weight. Even cutaneous lesions improved, whereas we documented only a partial benefit for peripheral arthritis. CVID could present with complicated phenotype and immune dysregulation, underlying a more complex syndrome, like CTLA-4 deficiency, thus suggesting the importance of genetic investigations in selected patients. Abatacept is a potential effective treatment in patients with documented CTLA-4 deficiency, in particular to induce and maintain remission of enteropathy

The ENERGY ECS Project: Smart and Secure Energy Solutions for Future Mobility

Electric and smart mobility are key enablers for their green energy transition. However, the electrification of vehicles poses several challenges, from the development of power components to the organization of the electric grid system. Moreover, it is expected that the smartification of mobility via sensors and novel transport paradigms will play an essential role in the reduction of the consumed energy. In response to these challenges and expectations, the ENERGY ECS project is pursuing smart and secure energy solutions for the mobility of the future, by developing power components, battery charging electronics, and self-powered sensors for condition monitoring, along with advanced techniques for grid management, applications of artificial intelligence, machine learning and immersing technologies. This paper presents the project’s objectives and reports intermediate results from the perspective of the targeted use cases