Real-Life Data on the Efficacy of Canakinumab in Patients with Adult-Onset Still's Disease.

BACKGROUND: Interleukin-1 inhibition has revealed to be a successful treatment approach for patients with adult-onset Still's disease (AOSD). However, real-life experience is focused on the use of anakinra, while data about canakinumab (CAN) are mainly based on case reports and small case series. Patients and Methods. Patients classified with AOSD according to Yamaguchi criteria and treated with CAN were consecutively enrolled. Their clinical and therapeutic data were retrospectively collected and statistically analysed to assess the role of CAN as a therapeutic opportunity in AOSD patients in terms of clinical and laboratory disease control along with corticosteroid-sparing effect. RESULTS: Nine AOSD patients (8 females and 1 male) treated with CAN for 15.00 ± 12.3 months were enrolled. Resolution of clinical manifestations was reported in 8/9 cases at the 3-month assessment; a significant decrease in the number of tender joints (p = 0.009), swollen joints (p = 0.027), and disease activity score on 28 joints-C-reactive protein (DAS28-CRP) (p = 0.044) was observed during the study period. The systemic score of disease activity significantly decreased at the 3-month and 6-month assessments and at the last visit compared to the start of treatment (p = 0.028, p = 0.028, and p = 0.018, respectively). The daily corticosteroid dosage was significantly reduced at the 3-month and at the last follow-up visits (p = 0.017 and p = 0.018, respectively). None of the patients experienced adverse events or severe adverse events during the follow-up. CONCLUSIONS: CAN has shown prompt and remarkable effectiveness in controlling AOSD activity in a real-life contest, with a significant glucocorticoid-sparing effect and an excellent safety profile.

Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases.

Monogenic autoinflammatory diseases (mAIDs) are inherited errors of innate immunity characterized by systemic inflammation recurring with variable frequency and involving the skin, serosal membranes, synovial membranes, joints, the gastrointestinal tube, and/or the central nervous system, with reactive amyloidosis as a potential severe long-term consequence. Although individually uncommon, all mAIDs set up an emerging chapter of internal medicine: recent findings have modified our knowledge regarding mAID pathophysiology and clarified that protean inflammatory symptoms can be variably associated with periodic fevers, depicting multiple specific conditions which usually start in childhood, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome, and mevalonate kinase deficiency. There are no evidence-based studies to establish which potential genotype analysis is the most appropriate in adult patients with clinical phenotypes suggestive of mAIDs. This review discusses genetic and clinical hints for an ideal diagnostic approach to mAIDs in adult patients, as their early identification is essential to prompt effective treatment and improve quality of life, and also highlights the most recent developments in the diagnostic work-up for the most frequent hereditary periodic febrile syndromes worldwide.

Longitudinal evaluation of two different COVID-19 vaccination strategies in individuals with and without previous SARS-CoV-2 infection.

Background: We tested the antibody response to SARS-CoV-2 vaccination in individuals with and without previous infection that received different vaccination strategies. Methods: We recruited 203 volunteers. Individuals who have had SARS-CoV-2 infection during the six months preceding vaccination received one dose (group 1), the others received two (group 2). After 3 months, 98 subjects received a booster dose. Anti-SARS-CoV-2 Spike RBD IgG were tested in all subjects before vaccination (T0), and at 15 (T15), 90 (T90), 180 (T180) and 360 (T360) days after second or single dose; additionally, in group 2, IgG were tested 10 days after the vaccination (T10). Results: The difference of IgG concentration between the groups was statistically significant (p<0.05) at T0, T15 and T90, but not at T180 (p=0.713) and T360 (p=0.069). At T0 and T90 the antibody titre was higher in group 1, but it dropped in all volunteers 90 days after vaccination. Most of infections after vaccination occurred between T90 and T180. Conclusions: Antibody titre is significantly associated with a previous SARS-CoV-2 infection. Probability of contracting the infection increases after three months from primary vaccination, even among who had a previous infection, confirming the efficacy of vaccination as a preventive measure against SARS-CoV-2 infections and the need of booster administrations.