Exploring relief for Behçet's disease refractory oral ulcers: a comparison of TNF inhibitors versus apremilast.

OBJECTIVES: Oral and genital ulcers are the hallmark manifestation of Behçet's disease (BD), significantly impacting patients' quality of life. Our study focuses on comparing the effectiveness and safety of TNF inhibitors (TNFis) and apremilast in controlling oral ulcers of BD, aiming to provide evidence-based guidance for physicians in selecting appropriate treatment modalities. METHODS: A retrospective analysis was performed on BD patients treated between December 2016 and December 2021 with TNFis or apremilast for refractory oral ulcers. The study assessed treatment response by the absence of oral ulcers at 3 and 6 months, with additional evaluations for genital ulcers and articular involvement. RESULTS: The study included 78 patients, equally allocated between TNFis and apremilast treatments. Both groups showed significant oral ulcer reduction at 3 (p< 0.001) and 6 months (p= 0.01) with no significant difference between the treatments. Apremilast had a notable corticosteroid-sparing effect by the 3-month follow-up, persisting through 6 months. Both treatments were equally effective in reducing genital ulcers, with TNFis showing greater effectiveness in addressing articular involvement. Apremilast had a higher discontinuation rate due to gastrointestinal side effects. CONCLUSION: TNFis and apremilast are both effective for treating BD refractory oral ulcers. While TNFis may offer broader benefits for other disease manifestations, apremilast is distinguished by its corticosteroid-sparing effect, especially for patients with a milder disease phenotype. Treatment selection should consider individual disease severity and clinical features to ensure a personalized and effective management strategy.

Changes in Cognitive and Functional Status and in Quality of Life of Older Outpatients during the COVID-19 Pandemic.

BACKGROUND: Older adults denoted one of the populations that mostly suffered from the consequences of the COVID-19 pandemic. The cost of confinement was paid in terms of social isolation, distance from relatives and friends, lack of social support, and limited access to the healthcare system, which had a negative impact on health of older adults with comorbidities and frailty. OBJECTIVES: The purpose of the present study was to report the consequences of the COVID-19 pandemic on cognitive performances, functional status, and health-related quality of life among frail outpatients, compared to pre-pandemic status. METHOD: The current sample was part of a larger sample of frail and pre-frail outpatients, who were first evaluated at the clinic between April and May 2019 and who underwent a first follow-up evaluation between April and May 2020. Those outpatients who have undergone the first follow-up evaluation were contacted between April and May 2021 and were asked to voluntarily participate in a second telephone-based evaluation. Cognitive performances (through Mini Mental State Examination - MMSE), functional independency in basic and instrumental daily activities, physical and mental components of health-related quality of life (SF-12 PCS and SF-12 MCS, respectively) were evaluated and compared to previous evaluations. RESULTS: Seventy one outpatients (mean age of 80.69 years) completed the present follow-up evaluation. Patients reported significantly lower cognitive performances (mean MMSE 19.37; p < 0.001), lower physical quality of life (mean score 31.69; p < 0.001), and lower mental quality of life (mean score 38.79; p < 0.001) compared to both pre-pandemic baseline and the first follow-up. Moreover, patients showed a significantly reduced independency in basic daily activities (mean score 3.8; p = 0.004), and a significantly reduced independency in managing telephone (p = 0.012) and medications (p = 0.035), compared to baseline. CONCLUSIONS: The COVID-19 pandemic has been a prolonged stressor over time, which has markedly affected health-related quality of life of outpatients, and it can be considered a stressor that might have contributed to the patients' greater cognitive and functional vulnerability.

Autoimmune hepatitis in genetic syndromes: A literature review.

Genetic syndromes represent relevant and rare diseases. These conditions include a large amount of epidemiological, pathogenetic and clinical features. However, a systematic approach to genetic syndromes is often prevented by the rareness of these diseases. So, although clinical features are usually precisely defined, nowadays more uncommon associations between genetic syndromes and internal medicine related diseases have been insufficiently studied. Autoimmune hepatitis (AIH) is a chronic liver disease caused by loss of tolerance to hepatocyte-specific auto-antigens. Conversely, a better knowledge about specific genetic syndromes in which AIH is more frequent could be important in the clinical management of patients, both for an early diagnosis and for a prompt therapy. Furthermore, a systematic approach could explain if onset, clinical course, and response to treatment of AIH are typical for specific genetic syndromes. We took in consideration all the scientific articles reported in PubMed in the last 10 years, from 2010 to 2020. The purpose of this review is to explore the prevalence of AIH in genetic syndrome, but also to suggest new classification, that could be useful for pathogenetic hypothesis and clinical approach to genetic syndrome. From the 139 publications selected using keywords "autoimmune hepatitis" and "genetic syndrome", 30 papers (21.6%) respected the chosen inclusion criteria, reporting the association between AIH in patients with a genetic syndrome. We have collected in all 47 patients with AIH and genetic syndrome, and with median age of 12.6-year-old. We suggest that when a patient presents a clinical picture of cryptogenic chronic hepatitis, that is unexplained, it is useful to explore differential diagnosis of AIH associated with genetic syndrome. Given the clinical relevance of this topic, further reports are needed to demonstrate our hypothesis and collect new evidence in this field.